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Hematology PPT Flashcards Unit 4

What is Hemostasis and Thrombosis? The maintenance of circulation hemostasis is achieved through the process of balancing bleeding (hemorrhage) and clotting (Thrombosis)

What is hemostasis? Prevent from bleeding or hemorrhageWhat is thrombosis? ClottingWhat are four components of hemostasis? – Vascular system

– Platelets (thrombocytes)– Blood coagulation factors– Fibrinolysis and ultimate tissue

repairWhat are three important component of protein in Hemostasis?

Complement system is a cascade produced in the liver.

Kinin systems plays a role in inflammation, coagulation, sodium homeostasis.

Protease inhibitorsWhat are several functionally processes are involved in hemostasis of a small blood vessel?

– Blood vessel spasm– Formation of a platelet plug– Contact between damaged blood vessel,

blood platelet, and coagulation proteins– Development of a blood clot around the

injury– Fibrinolytic removal of excess hemostatic

material to reestablish vascular integrityWhat is the function of Arteries? Blood away from heart, arteries have the

thickest walls of the vascular system.What is the function of Veins? Blood come into the heart, veins are larger and

haveMore irregular lumen than arteries

What is the function of capillaries? Gas exchangeWhat are the characteristics of Arterioles? Arterioles are the microscopic continuation of

arteries that give off branches called metarterioles, which in turn join the capillaries.

How were arterioles operated inside the capillaries?

The walls become thinner as the arterioles approach the capillaries, with the wall of a very small arteriole consisting only of an endothelial lining and some smooth muscle surrounded by a small amount of connective tissue.

How was the microcirculation formed? The capillaries, arterioles, and venules constitute the major vessels of the microcirculation.

What is the function of microcirculation? As a unit, the microcirculation functions as the link between the arterial and the venous circulations.

What is vasculature physiology role in hemostasis?

• The role of vasoconstriction in hemostasis

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• The role of the endothelium• Maintenance of vascular integrity

How does the vasoconstriction play a role in hemostasis?

When a smaller vessel, such as an arteriole, venule, or capillary, is injured, contraction occurs to control bleeding. This contraction of the blood vessel wall is called vasoconstriction.

What is the endothelium? The endothelium contains connective tissues such as collagen and elastin.

How does endothelium play a role in hemostasis?

The endothelium is highly active metabolically and is involved in the clotting process by producing or storing clotting components.

What was endothelium role in lysis of fibrin clots hemostasis?

It is also rich with plasminogen activator, which, if appropriately stimulated, is released and activates plasminogen, which ensures rapid lysis of fibrin clots.

What is the prostacyclin role in hemostasis? Additionally, the endothelium elaborates prostacyclin, which is synthesized by the endothelium from prostaglandin precursors and strongly inhibits platelet aggregation and adhesion.

How was the primary phase hemostasis activated after disruption of the endothelium?

Initially, rapid vasoconstriction for up to 30 minutes reduces blood flow and promotes contact activation of platelets and coagulation factors

How was the second phase hemostasis activated after disruption of the endothelium?

In the second phase, platelets adhere immediately to the exposed subendothelial connective tissue, particularly collagen. The aggregated platelets enhance sustained vasoconstriction by releasing thromboxane A2 and vasoactive amines, including serotonin and epinephrine

How was the third phase hemostasis activated after disruption of the endothelium?

In the third phase, coagulation is initiated through both the intrinsic and extrinsic systems

How was the final phase hemostasis activated after disruption of the endothelium?

Finally, fibrinolysis occurs following the release of tissue plasminogen activators (t-PAs) from the vascular wall. Fibrinolytic removal of excess hemostatic material is necessary to reestablish vascular integrity

How many factors are required to maintain of Vascular Integrity hemostasis?

Vascular integrity or the resistance to vessel disruption requires three essential factors

What are the essential factors of vascular integrity in hemostasis?

Circulating functional plateletsAdrenocorticosteroidsAscorbic acid

What will be happened if there is lack of A lack of these factors produces fragility of the

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essential factors in vascular integrity hemostasis?

vessels, which makes them prone to disruption.

What will be the initial series observation of megakaryocytic cell?

General characteristics of megakaryocytic development

What will be the second series observation of megakaryocytic cell?

The developmental sequence of platelets

What is the third series observation of megakaryocytic cell?

Cellular ultrastructure of a mature platelet

What will be the last series observation of megakaryocytic cell?

Platelet kinetics, life span, and normal values

How were the platelets (thrombocytes) role in hemostasis of megakaryocytic development?

Mature platelets (thrombocytes) (metabolically active cell fragments) are the second critical component in the maintenance of hemostasis

Where were the platelets (thrombocytes) found?

These anuclear cells circulate in the peripheral blood after being produced from the cytoplasm of bone marrow megakaryocytes, the largest cells found in the bone marrow

What are the stages of being formed platelets? 1) Megakaryoblast2) Promegakaryocyte3) Megakaryocyte

a. PlateletsWhere were the bone marrow megakaryocytes found?

Bone marrow megakaryocytes are derived from pluripotential stem cells

How were the platelets formed inside the cytoplasmic membrane?

The sequence of development from megakaryocytes to platelets is thought to progress from the proliferation of progenitors to polyploidization, that is, nuclear endoreduplication, and finally to cytoplasmic maturation and the formation of platelets

Where was the initial process of megakaryocytopoiesis take place?

Megakaryocytopoiesis proceeds initially through a phase characterized by mitotic division of a progenitor cell; Followed by a wave of nuclear endoreduplication.

What is endoreduplication process of progenitors?

Endoreduplication is the process in which chromosomal material (DNA) and the other events of mitosis occur without subsequent division of the cytoplasmic membrane into identical daughter cells

What are two classes of progenitors that have been identified in the early development of platelets?

1) Burst-forming-unit megakaryocyte (BFU-M)2) Colony-forming-unit megakaryocyte (CFU-

M)What is the function of Burst-forming-unit megakaryocyte (BFU-M)?

The BFU-M is the most primitive progenitor cell committed to megakaryocyte lineage

What is the second stage of megakaryocyte development of platelets?

The next stage of megakaryocyte development is a small, mononuclear marrow cell that

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expresses platelet-specific phenotypic markers but is not morphologically identifiable as a megakaryocyte

How well were the mononuclear marrow cells of platelets visible in cytoplasmic?

These transitional cells represent 5% of marrow megakaryocyte elements.

Have the transitional immature megakaryocyte cells been undergoing endomitosis?

Some transitional immature megakaryocyte cells may be capable of cellular division, but most are nonproliferating while actively undergoing endomitosis.

Described the final stage of megakaryocyte development:

The final stage of megakaryocyte development is the morphologically identifiable megakaryocyte.These cells are readily recognizable in the marrow because of their large size and lobulated nuclei. These cells are polyploid.

What is the size of a mature platelet? Platelets have an average diameter of 2 to 4 mm, with younger platelets being larger than older ones

Does a platelet have a nucleus? In contrast to megakaryocytes, platelets have no nucleus.

What is the characteristic of a platelet? The cytoplasm is light blue, with evenly dispersed, fine red-purple granules.

What is the characteristic of an inactive platelet?

An inactive or unstimulated platelet circulates as a thin, smooth-surfaced disc.

What is platelet adhesion? When vascular injury exposes the endothelial surface, underlying collagen platelets adhere to the subendothelial collagen fibers, spread pseudopods along the surface, and clump together (aggregate).

How quickly does platelet adhesion to subendothelial connective tissue, especially collagen happen?

1-2 minutes after a break in the endothelium.

What’s the best test to use to determine platelet function?

Platelet aggregation.

What is a thrombus? When the bridges formed by fibrinogen in the presence of calcium produce a sticky surface on platelets, resulting in aggregation and then reinforced by fibrin is considered a thrombus.

What substances can cause a blockage of a pathway by an aggregation of platelets?

Prostaglandin E (PG E), adenosine, and nonsteroidal anti-inflammatory agents (e.g., aspirin).

How are permanently anchored platelet plugs given additional consolidation and stabilization?

With fibrinogen, under the influence of small amounts of thrombin. This process involves the precipitation of polymerized fibrin around each platelet resulting in a fibrin clot that produces an

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irreversible platelet plug.Why is a platelet count a fundamental component in the evaluation of a patient?

Examination of the peripheral blood smear for platelet number and morphology is critical because manyclinical clues may be obtained from an evaluation ofplatelet quantity and morphology.

What are other fundamental evaluations of platelets?

Platelet aggregation, platelet adhesion, and antiplatelet antibody assays. Blood coagulation factors are one of the most important of the assays.

What are blood coagulation factors? Basic concepts of blood coagulation, common characteristics of coagulation factors, characteristics of individual factors, the mechanism of coagulation, fibrin formation, laboratory assessment of blood coagulation factors, and assays for fibrin formation.

What is blood coagulation a sequential process of?

Chemical reactions, involving plasma proteins, phospholipids, and calcium ions.

What are circulating factors that participate in the coagulation process designated by?

Roman numerals.

How are the roman numerals effected if the enzymatic factor is activated?

It’s a roman numeral followed by the suffix –a and the inactive enzymatic factor, zymogens are the roman numeral alone.

What are 3 assays that are conducted by specialized coagulation laboratories?

1) One-stage quantitative assay for factors II, V, VII, and X.2) One-stage quantitative assay for factors VIII, IX, XI, and XII.3) Factor VIII antibodies in hemophiliacs.

What specimen is used for the One-stage quantitative assay for factors II, V, VII, and X?

Aged serum and absorbed plasma in the PT.

What factors does age serum contain? Factors VII, IX, X, IX, and XII.What factors does absorbed plasma contain? Factors V, VIII, XI, and XIIIWho is the PT for One-stage quantitative assay for factors II, V, VII, and X conducted?

Using specific factor-deficient plasma with specific dilutions of patient plasma.

Why is the percentage of factor activity plotted?

To construct an activity curve.

For One-stage quantitative assay for factors VIII, IX, XI, and XII, what procedure is it based on?

The APTT assay.And it is based on the results of patient plasma to correct specific factor-deficient plasma.

Who are the results expressed in One-stage quantitative assay for factors VIII, IX, XI, and XII?

In percent activity on an activity curve.

What is the factor VIII antibodies in An ELISA technique that uses the binding of

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hemophiliacs assay? antibodies in the plasma to solid phase antigen, which is subsequently detected by a human polyclonal IgG labeled with the alkaline phosphatase-p-nitrophenyl phosphate substrate system.

What is Warfarin? The traditional oral anticoagulant (Coumadin). Warfarin drugs are vitamin K antagonists that interfere with the normal synthesis of factors II, VII, IX, and X as well as proteins C and S.

Why do Warfarin drugs cause incomplete coagulation?

Because they lack calcium-binding sites and

cannot form enzyme substrate complexes. Thus, these factors are unable to function as procoagulants or anticoagulants.

Biological activity is significantly1)______ (decreased or increased) as revealed by the PT.

Decreased

The onset of action of most warfarin derivatives is between how many hours?

8 and 12 hours

The maximum affect occurs in approximately how many hours?

36 hours

What is the duration of action of Warfain? Approximately 72 hours.What is the mainstay of immediate therapy for acute PE.

Heparin anticoagulation

How does Heparin acts as an anticoagulant? Heparin has no anticoagulant activity of its own but acts as an anticoagulant by accelerating the binding of antithrombin to target enzymes (e.g., thrombin and factor Xa).

Why is Heparin termed an antithrombin? Because it helps prevent new thrombus formation and buys time for endogenous fibrinolytic mechanisms to lyse the clot.

What can Heparin cause? Bleeding, thrombocytopenia, and osteopenia.Before initiating heparin, what should patients be screened for?

Clinical evidence of active bleeding

What should the baseline laboratory evaluation include?

CBC, platelets, PTT, PT, stool analysis for occult blood, and urine dipstick for hematuria.

When is Heparin anticoagulation used? During percutaneous transluminal coronary angioplasty (PTCA) and cardiopulmonary bypass (CPB) to prevent clot formation.

What are 2 other Antithrombin-Dependent inhibiters?

1) Danaparoid (Orgaran)– 2) Fondaparinux (Arixtra)

What 2 new types of drugs are direct thrombin inhibitors?

1) Hirudin, lepirudin (Refludan)2) Argatroban

What are the new types of thromboplastins for measuring the PT?

Mixtures of phospholipids and recombinantly derived human tissue factor.

What are Fibrinogen assays useful for? Detecting deficiencies of fibrinogen and

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alterations in the conversion of fibrinogen to fibrin.

The normal value of 200 to 400 mg/dL may be decreased in?

Liver disease or the consumption of fibrinogen owing to accelerated intravascular clotting

What is the normal titer of fibrinogen? 1:128 to 1:256.What does the thrombin time test determines? The rate of thrombin-induced cleavage of

fibrinogen to fibrin monomers and the subsequent polymerization of hydrogen-bonded fibrin polymers to form an insoluble fibrin clot.

What is the normal value of thrombin? The normal value is less than 20 seconds.What is the Reptilase time assay similar to? The thrombin time assay.What is the D-Dimer? Is a specific fragment generated from two cross-

linked fibrin molecules after a clot has formed.What are the 3 major hemostatic stages that must be functioning in order for D-dimers to form?

1) Coagulation to form the clot2) Covalent cross-linking of fibrin by activated factor XIII3) Fibrinolysis to dissolve the fibrin clot into smaller fragment

All 3 of these stages require adequate formation of?

Thrombin

What are 4 normal protective mechanisms against Thrombosis?

1) Normal blood flow2) Removal of activated clotting factors and particulate material3) The natural anticoagulant systems4) Cellular regulators

In the blood circulation, the predisposition to thrombosis depends on?

The balance between procoagulant and anticoagulant factors.

Several important biological activities normally protect the body against thrombosis. What are these activities?

-The normal flow of blood-The removal of activated clotting factors and particulate material-Natural anticoagulant systems known to be operative in vivo: * Antithrombin III (AT-III) * Heparin cofactor II (HC-II) * Protein C and its cofactor, protein S-Cellular regulators

What does the normal flow of blood prevent? The accumulation of procoagulant material.This mechanism reduces the chance of? Local fibrin formation.What is another normal mechanism against inappropriate thrombosis?

The removal of activated clotting factors by hepatocytes from the blood.

What does this process, along with the naturally occurring inhibitors do?

Limits intravascular clotting and fibrinolysis by inactivation of such factors as XIa, IXa, Xa, and IIa.

Why is the removal of particulate material by the cells of the mononuclear phagocytic

It prevents the initiation of coagulation.

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system also important?Why is the in vivo existence of natural anticoagulant systems essential?

To prevent thrombosis.

What are the natural anticoagulant systems? Antithrombin III, heparin cofactor II, and protein C and its cofactor, protein S

What are AT-III and HC-II? Serine-protease inhibitors.When activated, protein C is capable of degrading which activated factors?

V (Va) and VIII (VIIIa) in the presence of the cofactor protein S.

What is considered the major inhibitor of coagulation?

Antithrombin III (AT-III)

What are the serpin superfamily of serine proteinase inhibitors?

III (AT-III), alpha-1 antitrypsin, C1 inhibitor, alpha-2 antiplasmin, heparin cofactor II, and plasminogen activator inhibitor.

Heparin is produced endogenously by? Mass cells.Heparin-like molecules are found in the? Endothelium.What are the two heparin-dependent thrombin inhibitors present in human plasma?

AT-III heparin cofactor and heparin cofactor II, previously referred to as heparin cofactor A.

The inhibitory activity of heparin cofactor II is accelerated by?

Heparin.

What does the inhibition of thrombin by heparin cofactor II not limited to?

The activity of thrombin or fibrinogen; also inhibited are thrombin-induced platelet aggregation and release.

In addition to thrombin, heparin cofactor II also inhibits?

Chymotrypsin

Heparin cofactor II does not significantly inhibit?

Blood coagulation factors IXa, Xa, and XIa or plasmin.

What 2 proteins are involved in one of the major natural anticoagulation systems in the body?

Protein C and S

Deficiency and/or alteration in either protein (C and/or S) have been clearly associated with?

Predisposition to thrombosis.

What does Protein C (A vitamin K–dependent plasma protein synthesized in the liver) represents

A natural anticoagulant formed in response to thrombin generation.

What is APC resistance (A hypercoagulable condition discovered in 1993) is associated with

A point mutation in the factor V gene [factor V (Leiden)].

What does the mutation results in? The replacement of Arg506 with Gln(Q) in the factor V protein.

This mutation slows the inactivation of what factor causing a hypercoagulable state?

Factor Va by APC

What is the most common cause of The presence of factor V (Leiden)?

Inherited thrombophilia.

It accounts for ____? % to _____? % of the cases.

20% to 50%

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Protein S is another vitamin K–dependent plasma protein that is an essential cofactor for?

APC to express an anticoagulant effect.

Protein S does not require 1) ____? modification to function, but it can be regulated by 2) ____?

1) Proteolytic2) proteolysis.

What is becoming recognized as essential to the maintenance of hemostasis and thrombosis?

Cellular activities related to thrombosis

What are 2 powerful mechanisms to limit the formation or spread of clotting and the reliquefication of clots?

Plasma possesses, and plasmin

What have been demonstrated to be the major pathway for activation of coagulation?

Tissue factor and factor VIIa in the extrinsic pathway

What has also been discovered about thrombi?

That it has a far more important role as an activator and an inhibitor.

What is the key enzyme in the conversion of soluble fibrinogen into fibrin

Thrombin

What is required for the activation of the protein C system, which downregulates hemostasis?

Thrombin

What is required for the activation of thrombin-activatable fibrinolysis inhibitor (TAFI), which is involved in the downregulation of the fibrinolytic process?

Thrombin

What has led to the development of alternative schematic representations of the pathways that better represent the normal in vivo process of coagulation?

The cumulative result of new discoveries in coagulation has led.

What may cause abnormal bleeding? Disorders of the microcirculation, platelets, or plasma proteins.

What is purpura? Abnormal bleeding involving the loss of red blood cells from the microcirculation.

Purpura is characterized by? Hemorrhages into the skin, mucous membranes, and internal organs.

Purpura may be produced by a variety of vascular abnormalities such as?

1) Purpura associated with direct endothelial cell damage. Endothelial damage may result from physical or chemical injury to the tissue caused by microbial agents such as in rickettsial disease or immunological antibody-mediated injury. Bacterial toxins produce deendothelialization induced by an endotoxin. Antibody vascular injury, vasculitis, may be induced by drug reactions, insect bites, or the activation of complement.2) Purpura associated with an inherited disease of the connective tissue. Alterations of the vascular supportive framework can occur in disorders such as diabetes.3) Purpura associated with decreased

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mechanical strength of the microcirculation. Decreased strength can be seen in conditions such as scurvy and amyloidosis.4) Purpura associated with mechanical disruption of small venules. The principal cause of this type of purpura is increased intraluminal pressure. This condition can be observed around the ankles with prolonged standing and may be caused by the presence of abnormal proteins in macroglobulinemias or hyperviscosity disorders.5) Purpura associated with microthrombi (small clots). This type of disorder is associated with abnormal intravascular coagulation conditions.6) Purpura associated with vascular malignancy. Purpura of this origin is observed in Kaposi sarcoma and vascular tumors.

What are the disorders of platelet size? 1) Wiskott-Aldrich syndrome 2) May-Hegglin anomaly3) Alport syndrome 4) Bernard-Soulier syndrome

What is Wiskott-Aldrich syndrome? Is the smallest platelets seen.

What is May-Hegglin anomaly? It is characterized by the presence of large platelets and the presence of Döhle-like bodies in the granulocytic leukocytes.

What is Alport syndrome? Exhibits giant platelets and thrombocytopenia

What is Bernard-Soulier syndrome? Is the largest platelets seen and is also referred to as giant platelet syndrome.

What is the normal range of circulating platelets?

150 x 109/L to 450 x 109/L

When the quantity of platelets decreases to levels below 150 x 109/L to 450 x 109/L a condition known as _____? exists.

Thrombocytopenia

Is the quantity of platelets increases then what is the result?

Thrombocytosis

Disorders of platelets can be classified as? Quantitative (thrombocytopenia or thrombocytosis) or qualitative (thrombocytopathy).

If platelets are absent or severely decreased below 100 x 109/L, what clinical symptoms are usually seen?

The presence of petechiae or purpura.

What is Petechiae? Small purplish hemorrhagic spots on the skin or mucous membranes.

What is Purpura? It is characterized by extensive areas of red or dark-purple discoloration.

Most thrombocytopenic conditions can be classified into major categories such as?

1) Disorders of production of platelets may be caused by hypoproliferation of the megakaryocytic cell line or ineffective

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thrombopoiesis caused by acquired conditions or hereditary factors.2) Disorders of destruction, including decreased megakaryocytopoiesis and ineffective platelet production, and disorders of utilization. It may result from a number of mechanisms—drugs, bacterial sepsis, etc.3) Disorders of platelet distribution and dilution

What is the most drug-induced thrombocytopenia?

Heparin-Induced Thrombocytopenia (HIT)

What are the predominant clinical symptoms of HI?

Thrombocytopenia and thrombosis

What are the two types of HIT that exist? 1) Nonimmune HIT: Type I2) Immune HIT: Type II

What replace the term idiopathic thrombocytopenic purpura?

New standard nomenclature, immune thrombocytopenia (ITP)

What is immune thrombocytopenia (ITP)? ITP is an acquired immune-mediated disorder characterized by isolated thrombocytopenia (platelet count <100 x 109/L) and the absence of any obvious initiating and/or underlying cause of the thrombocytopenia.

What can contribute to the increased utilization of platelets?

Intravascular coagulation, vascular injury or occlusion, and tissue injury.

What rapidly consumes platelets? DIC (disseminated intravascular coagulation)What are some examples of disorders that can trigger the accelerated consumption of platelets?

Trauma, obstetrical complications, and microbial sepsis.

What can contribute to the thrombocytopenia?

In the case of bacterial sepsis, thrombin-induced platelet aggregation in vivo contributes to thrombocytopenia.

Why do vascular injuries (vasculitis) causes a decrease in platelets?

Because of the direct consumption of platelets at the sites of endothelial loss without appreciable depletion of clotting factors such as fibrinogen.

What is thrombotic thrombocytopenic purpura (TTP)?

Is a clinical syndrome with a high mortality rate that is characterized by formation of microthrombi in the microvasculature.

What are the diagnostic of the diseases TTP? The hematological findings of thrombocytopenia and red blood cell schistocytes.

In TTP, coagulation testing will demonstrate 1) ____ prothrombin and 2) _____ partial thromboplastin time but 3) ______D-dimer and fibrinogen levels.

1) Normal2) Activated3) Elevated

TTP is in contrast to DIC, which demonstrates 1) _____ prothrombin time and 2) _____ partial thromboplastin time.

1) Abnormal2) Activated

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What 3 types of TTP have been identified? 1) Idiopathic2) Secondary3) Inherited (Upshaw-Shculman)

A platelet distribution disorder can result from?

A pooling of platelets in the spleen, which is frequent if splenomegaly is present.

This type of thrombocytopenia develops when?

More than a double or triple increase in platelet production is required to maintain the normal quantity of circulating platelets.

Disorders that may produce splenomegaly with resultant splenic pooling or delayed intrasplenic transit include?

Alcoholic or posthepatic cirrhosis with portal hypertension, lymphomas, and leukemias and lipid disorders, for example, Gaucher disease.

Generally defined as a substantial increase in circulating platelets over the normal upper limit of 450 x109/L. Thrombocytosis can be classified into three major categories which are?

1) Hereditary or familial thrombocytosis associated with germline mutations of the thrombopoietin (THPO) gene in the thrombopoietin receptor (MPL) gene2) Thrombocytosis associated with myeloproliferative neoplasms and/or myelodysplastic disorders (clonal thrombocytosis associated with somatic mutations of JAK2 [V617F], MPL, and additional currently unknown genes)3) Reactive (secondary thrombocytosis)

If platelets are normal in number but fail to perform effectively then….?

A platelet dysfunction exists.

In addition to both individual and family medical histories, laboratory tests are critical for?

Determining a platelet dysfunctional diagnosis.

What do Laboratory tests of platelet function include?

bleeding time, clot retraction, platelet aggregation, platelet adhesiveness, and antiplatelet antibody assay.

Three separate categories of platelet dysfunction can be identified based on?

Etiology- the more common acquired causes and the less frequent hereditary causes.

Hyperactive platelets associated with hypercoagulability and thrombosis make up an additional category of what?

Abnormal platelet function.

Examples of inherited disorders include? Bernard-Soulier syndrome and Glanzmann thrombasthenia.

What is responsible for the initial phases of hemostasis.

Vascular response and platelet plug formation.

Subsequent to these activities what is initiated to form the fibrin clot?

The clotting factors

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Fibrin formation can occur if ? The activity of various factors is at least 30% to 40% of normal.

Bleeding and defective fibrin clot formation are frequently related to what?

A coagulation factor

Disorders of the blood coagulation factors can be grouped into three categories, which are?

1) Defective production may be related to a deficiency of vitamin K. Classic hemophilia (hemophilia A) and von Willebrand disease are examples of hereditary disorders that represent functionally inactive factor VIII.2) Excessive destruction3) Pathological inhibition

What is hemophilia ? When a clotting factor is either missing or is not working to its full ability causing for a greater chance of prolonged bleeding as well as the blood taking longer to clot

Is there a cure for hemophilia No cure is a life long disease but can be treated and managed

What causes hemophilia ? It is an X-linked autosomal recessive disease this means that the women are the carries and the men are affected

What is hemophilia A? Clotting factor 8 is either deficient or not presentWhat is hemophilia B? Clotting factor 9 is deficient or not presentWhat is Von willebrand Disease ? This is when vonon willebrand clotting

factor is not present this can affect both men and women

What is mild severity of hemophilia? 6%-50% of the clotting factor is activeWhat are manifestation of hemophilia ? Hemarthrosis- bledding in the joint

Hematuria- blood in the urineEcchymosis-bruising

What types of tests are done to diagnose hemophilia?

Partial thromboplastin time (ptt)Typically prolongedProthrombin time (pt)Bleeding timeFibrinogen levelPlatelet countFactor 8,9 and van willebrand assayGenetic testing

True or false ? von willebrand disease may be an acquired or inherited disorder ?

True

What distinguish von willebrand disease from classic factor V111: C ?

A deficiency of factor V111 procoagulant activity

Von wildebrand’s disease occurs in ____of population

1-2%

_________is a major relatory mechanism of hemostasis

Protein C (PC)

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Pts who have herediatary protein Cor S deficiency have of what if take Coumadin?

Skin necrosis

Is homozygous antithrombin deficiency compatible with life?

No

What is DIC ? DIC is a complication or intermediary phase of many diseases and does not constitute a disorder in itself

What include triggering events that may predispose patients to DIC ?

Alterations in the endothelium direct activation of fibrinogen release of thromboplastin-like substances and erythrocyte or platelet destruction

What are some examples of clinical situations in which tissue thromboplastin can activate coagulation ?

Extravascular trauma abruption placentae advanced malignancy leukemia and retained fetal synf=rome

Through what 4 ways does the body stop the clotting process ?

TFPIantithrombin IIIProtein C / Protein S /ThrombomodulinPlasminogen

How does Heparin help inhibit the clotting process ?

catalyzes antithrombin III's inactivation of IX, X, XI, and thrombin

How does TFPI (Tissue Factor Pathway Inhibitor) work ?

complexes with factors VIIa, TF, and Xa, and inactivates Xa

How does fibrinolysis happen ? TPA (Tissue Plasminogen Activator), which is on the surface of endothelial cells, converts plasminogen--> plasmin. Plasmin is a general protease that loves blasting proteins w/lysine residues. Fibrin has a lot of lysine residues, so it looks particularly sexy to plasmin (high affinity). It turns what was once a cold-hearted insoluble clot all soluble and easygoing, and D-dimers float around.

What is disseminated intravascular coagulation

A life-threatening uncontrolled activation of the coagulation system

What are some characteristics of DIC 1. Widespread small vessel thrombosis2. Consumption of platelets and factors3. Uncontrolled bleeding due to depletion of platelets and coagulation factors4. organ failure

What is DIC caused by? Various clinical conditions that activate clotting mechanisms:InfectionsHemorrhageShockSeptic shock

Is DIC a primary or secondary? DIC is never a primary condition. It is always secondary to another problem (ex: a person in septic shock would have DIC as a consequence

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Describe the onset and end result of DIC Stimulation of clottiing --> Thrombosis emboli formation --> Vascular occlusion --> Organ/tissue ischemia --> Infarction --> Necrosis

Why is DIC considered a consumptive colaguopathy ?

Because there's a primary stimulus that stimulates the body to think that it has a major issue where it needs to form clots (may be true in some cases for a trauma patient that is actively bleeding)As the small clots consume coagulation proteins and platelets, normal coagulation is disrupted and abnormal bleeding occurs from the skin

What does consumption of coagulation factors and platelets result in?

Bleeding

Why does hemorrhage occur? Because platelets and coagulation factors are no longer present

What occurs in all preestablished clots? Clot is programmed to break down and Fibrinolysis starts

How type of cycle is DIC? Vicious cycle of clotting and bleeding

What are two abnormal coagulation profiles that decrease?

Thrombocytopenia (decrease in platelets)andDecreased clotting factors (any Roman numeral, fibrinogen, or thrombin)

What are two abnormal coagulation profiles that increase?

Increased clotting measurements/ amount of time (Prothrombin time, Partial prothrombin time, bleeding times)andIncreased Fibrin Degradation Products (FDPs) or Fibrin Split Products (FSPs)-->D-Dimer is specifically measurable with FDP to indicate Fibrinolysis

How should DIC be evaluated? Monitor CBC for evolving and worsening anemia*The anemia will normally be Normocyctic normochromic from acute hemorrhage situation

What are some treatments for DIC? What are some treatments for DIC?

Remove percipitating primary) problem (because DIC is secondary consequenceRestore balance between coagulation and fibrinolysisMaintain organ viability Administer anticoagulants (heparin)

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What is responsibility of hematology technicians and technologists?

Gross physical examination, total cell count, microscopic examination, and other special tests

Where are chemical analyses and microbial and cytological examinations?

Examinations are generally performed in the chemistry, microbiology, and cytology departments, respectively

Where sterile body fluid can be found? It found in various body cavities under normal conditions. In diverse disorders and disease processes, the quantity of these fluids can increase significantly. Fluid specimens aspirated from different anatomical sites and examined for the total number of cells, differentiation of cell types, chemical composition, and microbial contents.

How all body fluids are should be handled with caution?

Standard precautions must be practiced.

What are the type of examination performed on the body fluid depends on?

the body fluid depends on the source of the specimen. However, a portion of the examination of cerebrospinal fluid (CSF); serous fluids from the pleural, pericardial, and peritoneal cavities; synovial fluid; and seminal fluid is frequently performed in the hematology laboratory.

What the most is commonly analyzed body fluid?

Cerebrospinal Fluid

How cerebrospinal fluids (CSF) are acts? CSF acts as a shock absorber for the brain and spinal cord, circulates nutrients, lubricates the central nervous system (CNS), and may also contribute to the nourishment of brain tissue.

Where cerebrospinal fluids (CSF) are circulates through?

The CSF circulates through the ventricles and subarachnoid space that surrounds both the brain and the spinal cord. The ventricles consist of

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four hollow, fluid-filled spaces inside the brain. A lateral ventricle lies inside each hemisphere of the cerebrum

What CSF is production? Primarily a function of the choroid plexus, with a smaller proportion being derived from the ependymal lining and perivascular spaces

What the plexus is composed of two layers? The ependyma (the lining epithelium of the ventricle) and the pia mater. The plexuses in the lateral ventricles are the largest and produce most of the CSF.

What make constitute the anatomical structure of the blood-brain barrier?

The choroid plexus epithelium and the endothelium of capillaries in contact with CSF.

Where CSF is found? Inside all the ventricles, in the central canal of the spinal cord, and in the subarachnoid space around both the brain and the spinal cord.

How many the total maximum volume of CSF in adults is?

About 150 mL. The maximum volume in neonates is approximately 60 mL.

How to measure CSF? Introducing a needle into the subarachnoid space makes it possible to measure CSF pressure and to obtain fluid for analysis.

How a specimen of CSF is examined? A specimen of CSF is examined visually and microscopically.The total number of cells can be enumerated, and the types of cells can be morphologically distinguished. .Gross physical examination

• Turbidity• Color• Viscosity• Microscopic examination: cellular

enumeration

What normal CSF is contains? A few mononuclear cells (lymphocytes and monocytes) and rare ependymal cells. Very few leukocytes should be seen in normal CSF. The generally accepted total number of WBCs in the CSF ranges from 0 to 5 cells/mL or 0 to 5 × 106/L. A value of up to 20 x106/L can be considered within the range of normal for children and adults. Neonates have a higher normal range, 0 to 30 cells × 106/L.

How many WBC count are low or high? Occasionally, the WBC count may be very low (0 to 20 × 106/L), but the CSF is turbid because of a high concentration of bacteria. In acute, untreated bacterial meningitis, the CSF leukocyte count usually ranges from 100 to

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10,000 cells × 106/L. Very high WBC counts (greater than 50,000 × 106/L) are unusual and suggest intraventricular rupture of a brain abscess.

Cells Unique to the Cerebrospinal Fluid Ependymal cellsChoroidal cells

Body fluids that are analyzed in the hematology laboratory.

Pleural, Peritoneal, and Pericardial Fluids

What is an effusion? An effusion is an abnormal accumulation of fluid in a particular space of the body. Effusions in the pleural, pericardial, and peritoneal cavities are divided into transudates and exudates.

What transudates generally? Fluid has accumulated because of the presence of a systemic disease.

What exudates are? Associated with disorders such as inflammation, infection, and malignant conditions involving the cells that line the surfaces of organs (e.g., lung or abdominal organs).

What is pleural fluid? normally produced by the parietal pleura and absorbed by the visceral pleura as a continuous process. Although healthy individuals form 600 to 800 mL of fluid daily, the normal volume of fluid in each pleural space is estimated at less than 10 mL.Aspiration of pleural fluid is referred to as thoracentesis.

How difference color between transudates and exudates?

Transudates are usually clear, are pale yellow, and do not clot. In comparison, exudates can display a range of colors depending on the associated disorder.

Erythrocyte and leukocyte counts They are of limited value in the differential diagnosis of pleural effusions. A massively bloody (hemorrhagic) effusion in the absence of trauma almost always suggests malignancy, or occasionally pulmonary infarct.

How WBC count are effect transudates and exudates

Extremely elevated total leukocyte (WBC) counts of 50.0 × 109/L or higher are consistent with a diagnosis of empyema. In general, WBC counts less than 1.0 × 109/L are associated with transudates, and WBC counts greater than 1,000 × 109/L are associated with exudates.

Cell types that can be encountered in the examination of a Wright-Giemsa–stained specimen include the following:

Neutrophils (PMNs)EosinophilsBasophilsLymphocytesPlasma cells

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Mononuclear phagocytes (monocytes, histiocytes, and macrophages)Mesothelial cells (normal, reactive, atypical, and malignant)Metastatic tumor (malignant) cells

What is peritoneal Fluid? A body fluid that is analyzed in the hematology laboratory.

How about an abnormal amount of fluid (an effusion)?

It can accumulate in the peritoneal cavity if the balance between fluid formation and reabsorption is altered by a disease process.

What are result of the collection of fluid in the peritoneal cavity, ascites?

Results from increased hydrostatic pressure in the systemic circulation, increased peritoneal capillary permeability, decreased plasma oncotic pressure, or decreased fluid reabsorption by the lymphatic system.

What are causes of Peritoneal Effusions? The causes of peritoneal effusions range from disorders and diseases that directly represent involvement of the peritoneum, such as bacterial peritonitis, to abdominal conditions that do not directly involve the peritoneum, such as hepatic cirrhosis, cirrhosis, congestive heart failure, Budd-Chiari syndrome, hypoalbuminemia (caused by nephrotic syndrome or protein-losing enteropathy malnutrition), and miscellaneous disorders such as myxedema, ovarian diseases, pancreatic disease, and chylous ascites.

How can effusions that may conform with the definition of transudates?

Can be associated with congestive heart failure, hepatic cirrhosis, and hypoproteinemia.

What the laboratory are criteria for distinguishing transudates from exudates?

less clearly defined for peritoneal (ascitic) fluid than for pleural fluid.

How difference baboratory Analysis of transudates and exudates?

Transudates are usually clear and pale yellow. Exudates are cloudy or turbid because of an increased concentration of leukocytes, elevated protein levels, and occasionally microorganisms. Exudates may be seen in peritonitis, cases of perforated or infarcted intestine, and pancreatitis.

What includes of an evaluation of ascitic fluid in?

gross inspection, total cell count, microscopic examination of sediment for cell differentiation, cytological studies, chemical analysis for constituents such as total protein and lactic dehydrogenase, and microbial culture.

What is total WBC count higher than 0.3 × 109/L?

Total WBC counts are of limited value in differential diagnosis, but a total WBC count

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higher than 0.3 × 109/L is considered to be abnormal.

What is A wide variation in the peritoneal WBC count?

It seen in patients with chronic liver disease because of extracellular shifts in fluid associated with ascites formation or resolution.

An abnormal accumulation of fluid in the cavity, a ?, is most frequently caused by damage to the lining of the cavity and increased capillary permeability.

pericardial effusion

In addition, in acute pericarditis, interference with ? venous and lymphatic drainage predisposes the patient to effusion development.

pericardial

A wide variety of diseases and disorders can produce ? effusion.

pericardial

Normal fluid is 1 and 2. Hemorrhagic (bloody) effusions may result from a variety of abnormal conditions or from aspiration of intracardiac blood into the specimen.

1. Transparent2. pale yellow

On visible examination, a hemorrhagic effusion should not form clots in a plain (nonanticoagulant) tube, but aspirated blood usually exhibits clotting. A milky-appearing effusion may be a true or ? fluid.

pseudochylous

Erythrocyte and leukocyte cell counts are of limited value in the differential diagnosis of a ?.

pericardial effusion

A ? is a body fluid that is analyzed in the hematology laboratory.

Seminal fluid

Seminal fluid (semen) is examined 1 and 2. 1. macroscopically2. microscopically

Semen analysis is the primary test for the evaluation of male infertility. Sperm concentration, motility, and morphology can be used to classify men as what other 3 categories

subfertile, of indeterminate fertility, or fertile.

? also can be analyzed for a variety of reasons, including artificial insemination protocols, postvasectomy assessment, and evaluation of probable sexual assault.

Semen

A fresh specimen is needed. It is mandatory that the specimen be kept at ?°C and examined within 1 hour of collection. After 60 minutes of storage in a plastic container, sperm motility is significantly reduced.

37°C

Is the following macroscopic or microscopic? Macroscopic

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A fresh specimen should be examined for coagulum formation, color, pH, volume, and viscosity.Is the following macroscopic or microscopic?Enumeration of the number of sperms and examination of the morphological characteristics of the cells are routinely performed procedures. Other microscopic procedures include motility, viability, and agglutination studies.

microscopic

What type fluid is a transparent, viscous fluid secreted by the synovial membrane. This fluid is found in joint cavities, bursae, and tendon sheaths

Synovial (joint)

Arthrocentesis constitutes a liquid biopsy of the ?.

joint

Disorders such as gout, calcium pyrophosphate dihydrate (CPPD) deposition disease, and septic arthritis can be diagnosed definitively by synovial fluid analysis and may allow for consideration or exclusion of rheumatoid arthritis and ?

systemic lupus erythematosus (SLE).

Synovial fluid is readily obtained by ? from most joints. Frequent sites of ? include the knee, shoulder, elbow, wrist, interphalangeal joints, hip, and ankle.

aspiration

Routine analysis of synovial fluid should include microscopic examination of a wet preparation, crystals, Gram stain, and ? culture.

microbiological

If the fluid is very turbid, or if septic arthritis is considered for other reasons, the specimen should be sent for 1. And 2.?

1. Gram stain2. culture

True or False? A Gram stain is needed if a high likelihood of infection exists.

– True

Other observations or procedures can include volume and appearance, viscosity, mucin test, chemical analysis for protein, and glucose. This is what type of examination?

– Gross

Normal, noninflammed joints have small quantities of clear or transparent fluid. This fluid is viscous and slightly alkaline and, if normal, does not clot. This describes what?

– appearance

The best use of the ? test is to distinguish the anatomical origin of bloody or other fluids.

– mucin clot

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The presence of a mucin clot implies that it is synovial fluid.A differential cell count on a body fluid should be performed on stained smears prepared from a concentrated preparation—not in a ?. Some of the techniques of sediment preparation and staining are different for body fluids than for blood.

– hemocytometer

Morphological descriptions of cells encountered in body fluids reflect their microscopic appearance with 1. or 2?

1. Wright2. Wright-Giemsa stain.

The coloration of cells with Papanicolaou stain is somewhat different. Papanicolaou stain is a commonly used what type of stain?

• Cytological

The following describe what type of format?• Procedure title and specific method• Test principle including type of

reaction and the clinical reasons for the test

• Specimen collection and preparation• Reagents, supplies, and equipment• Calibration of a standard curve• Quality control• Procedure• Calculations• Reporting results (normal values)• Procedure notes including sources of

error, clinical applications, and limitations of the procedure

• References

CLSI Pricedural Format

T or F? All specimens should be treated with caution. All blood, tissues, and blood derivatives should be considered potentially infectious.

True

A stained smear is examined to determine the percentage of each type of leukocyte present and assess the erythrocyte and platelet ?.

morphology

Increases in any of the normal leukocyte types and the presence of immature leukocytes or erythrocytes in peripheral blood are important diagnostically in a wide variety of inflammatory disorders and ?

leukemia.

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Erythrocyte abnormalities are clinically important in various ?.

anemias.

Platelet size irregularities are suggestive of particular what type of disorders?

thrombocyte

Disorders associated with increases or decrease in normal leukocyte types?

•• Neutrophils

Bacterial infections, inflammation, stress, and chronic leukemia

• LymphocytesViral infections, whooping cough, and

chronic leukemia• Monocytes

Tuberculosis, rheumatoid arthritis, and fever of unknown origin

• EosinophilsActive allergies and invasive parasites

• BasophilsUlcerative colitis and hyperlipidemia

Increase

The packed cell volume (PCV) is a measurement of the ratio of the volume occupied by the RBCs to the volume of whole blood in a sample of ?.

capillary or venous blood

Following centrifugation, this ? is measured and expressed as a percentage or decimal fraction.

ratio

The ? is used to calculate the mean corpuscular volume (MCV) and the mean corpuscular hemoglobin concentration (MCHC).

PCV

True or False? The PCV is used to detect anemia, polycythemia, hemodilution, or hemoconcentration.

True

Name the 11 elements that the CLSI Procedural Format must have.

• Procedure title and specific method• Test principle• Specimen collection and preparation• Reagents, supplies, and equipment• Calibration of a standard curve• Quality control• Procedure• Calculations• Reporting results (normal values)• Procedure notes

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• ReferencesWhat must be included in Test Principle? • Type of reaction and Clinical reasons for the

testWhat must be included in the Procedure Notes?

• Sources of error, clinical applications, and limitations of the procedure.

How must all specimens be treated? • With Caution

What specimens should be considered potentially infectious?

• All blood, tissues, and blood derivatives.

What are the 5 basic procedures in Hematology?

• Leukocyte differential count• Packed cell volume of whole blood• Red blood cell indices• Reticulocyte count

• Sedimentation rate of erythrocytesWhat is the principle of Leukocyte Differential Count?

To determine the percentage of each type of leukocyte present and assess the erythrocyte and platelet morphology

Increases in any of the normal leukocyte types and the presence of immature leukocytes or erythrocytes in peripheral blood are important diagnostically in what?

A wide variety of inflammatory disorders and leukemia.

What are erythrocyte abnormalities are clinically important in?

Various anemias

Platelet size irregularities are suggestive of what?

Thrombocyte disorders

Disorders associated with Neutrophils are? Bacterial infections, inflammation, stress, and chronic leukemia

Disorders associated with Lymphocytes are? Viral infections, whooping cough, and chronic leukemia

Disorders associated with Monocytes are? Tuberculosis, rheumatoid arthritis, and fever of unknown origin

Disorders associated with Eosinophils are? Active allergies and invasive parasitesDisorders associated with Basophils are? Ulcerative colitis and hyperlipidemiaHow to you read a slide when doing a differential count?

Up, Across, Down, Across

What is packed cell volume (PCV)? A measurement of the ratio of the volume occupied by the RBCs to the volume of whole blood in a sample of capillary or venous blood

How is the ratio is measured and expressed? As a percentage or decimal fraction.What does the PCV calculate? The mean corpuscular volume (MCV) and the

mean corpuscular hemoglobin concentration (MCHC).

What are the PCV Reference values? Males, 0.47 L/L ± 0.07Females, 0.42 L/L ± 0.05

What is the PCV is used to detect? Anemia, polycythemia, hemodilution, or hemoconcentration

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What are the 3 calculations of RBC Indices? Mean corpuscular volumeMean corpuscular hemoglobinMean corpuscular hemoglobin concentration

What does Mean Corpuscular Volume (MCV) do?

It expresses the average volume (size) of an erythrocyte

What is the MCV Reference value? 80 to 96 fLWhat does Mean Corpuscular Hemoglobin (MCH) do?

It expresses the average weight (content) of hemoglobin in an average erythrocyte.

What is MCH directly proportional to? The amount of hemoglobin and the size of the erythrocyte.

What is the MCH Reference value? 27 to 32 pg

What does Mean Corpuscular Hemoglobin Concentration (MCHC) do?

It expresses the average concentration of hemoglobin per unit volume of erythrocytes.

What does the MCHC defined as the ratio of? The weight of hemoglobin to the volume of erythrocytes.

What is the MCHC Reference value? 32% to 36%How do you calculate MCHC? Hemoglobin (g / dL)MCHC g / dL

PCV or hematocrit (L / L)

What is a reticulocyte? An erythrocyte still possessing RNAWhat is Renumeration of reticulocytes important in?

Assessing the status of erythrocyte production in the bone marrow (erythropoiesis).

What type of stain is new methylene blue N? Supravital stainsWhat does new methylene blue N cause? Ribosomal and residual RNA to co-precipitate

with the few remaining mitochondria and ferritin masses in living young erythrocytes to form microscopically visible dark-blue clusters and filaments.

What is Reticulum? Clusters and filaments.What is the Reference value of Reticulocytes in methyl blue?

0.5% to 1.5%; neonates, 2.5% to 6.5%

Name 2 disorders associated with abnormal reticulocyte results.

Decreased reticulocytes - Aplastic anemiaIncreased reticulocytes - Acute blood loss

What does Sedimentation rate measures? The rate of settling of erythrocytes in diluted human plasma

What does Sedimentation rate depend on? An interrelationship of variables, such as the plasma protein composition, the concentration of erythrocytes, and the shape of the erythrocytes.

What is the clinical value of this procedure? It is in the diagnosis and monitoring of inflammatory or infectious states.

What are 2 other names for Sedimentation Rate of Erythrocytes?

ESR and Sedrate

What is ESR directly proportional to? The weight of the cell aggregate and inversely proportional to the surface area.

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Which sediments more slowly, microctytes or macrocytes?

Microcytes

Name 3 factors that contribute to decrease in ESR.

The removal of fibrinogen by defibrination Erythrocytes with abnormalitieso Erythrocytes with irregular shapes

Name 3 irregular erythrocyte shapes. Sickle cells Spherocytes Hinder rouleaux

When is an increased ESR value seen various abnormal blood conditions: rouleaux, increased fibrinogen levels, a relative increase of plasma globulins caused by the loss of plasma albumin, and an absolute increase of plasma globulins.

What are some clinical conditions associated with increased ESR values?

a) Anemia, infections, inflammation, tissue necrosis, pregnancy, and some types of hemolytic anemia

Give an example of tissue necrosis. b) Myocardial infarction.Name 10 Specialized hematology procedures. • Acidified serum lysis test

• Bone marrow examination• Donath-Landsteiner screening test• Glucose-6-phosphate dehydrogenase activity

in erythrocytes• Hemoglobin electrophoresis• Hemoglobin S screening test• Malarial smears• Monospot test• Osmotic fragility of erythrocytes• Sucrose hemolysis test

What test is Acid serum lysis called? HAM testHow do you test for hemolysis? Erythrocytes are incubated with fresh and heated

serumWeak acid is used in specific serum cell mixtures to maximize what?

Hemolytic activity.

The presence of hemolysis may be observed in cases such as?

Antibody-sensitized coated erythrocytes, spherocytes, or paroxysmal nocturnal hemoglobinuria (PNH).

How is a marrow examined histologically? By using a hematoxylin-eosin (H&E) stain.Who preforms a bone marrow aspiration? A physicianWhat does the physician examine in the bone marrow?

Cellular activities of the marrow

What Stain(s) is used on bone marrow examinations?

Wright-Giemsa stainPrussian blue and cytochemical stains

What is bone marrow examination? Is valuable in the diagnosis of disorders and in the study of leukemia’s and some types of anemia specifically involving the marrow

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Give an example of a bone marrow examination.

Multiple myeloma.

What's the normal distribution of bone marrow cells in an adult?

Rubricytes, neutrophils, lymphocytes, monocytes, megakaryocytes, reticulum cells, plasma cells, myeloid, erythroid

What is the myeloid:erythroid ratio? M/E ratio 2.5:1The Donath-Landsteiner screening antibody test requires?

Cold incubation to exhibit hemolysis in the patient's serum.

A positive result shows? Paroxysmal cold hemoglobinuria(PCH), the rarest form of autoimmune hemolytic anemia

True or False: The observed rate of appearance of bright fluorescence is proportional to the blood G6PD activity.

TRUE

What is one of the most prevalent hereditary erythrocyte enzyme deficiencies?

Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency

A deficiency of G6PD can produce? Drug or stress induced hemolytic anemiaThe movement of charged particles on various media under the influence of an electric current is called?

Electrophoresis

True or False: Particles move at the same speeds because of their weight and electric charge.

FALSE

How is hemoglobin electrophoresis useful? To identify and quantify hemoglobin variants and abnormal quantities of hemoglobin fractions

A hemolysate prepared from intact erythrocytes is placed on which medium?

Cellulose acetate

What describes a biphasic system with an upper organic phase of toulene and a lower aqueous phase of phosphate buffer, saponin, and reducing agents?

Hemoglobin S Screening Test

How are erythrocytes lysed? By toluene and saponinThe released hemoglobin is reduced by? Sodium hydrosulfiteThe resulting colors of the aqueous phase and the interface phase allow for the differentiation of which hemoglobin types?

AA, AS, and SS

True or False: Detection of the abnormal Hb S is diagnostic of sickle cell disease.

TRUE

The detection of this heterozygous state is important to which individuals?

Long distance runners, test pilots

Thick and thin blood smears are prepared, stained and examined microscopically for which malaria types?

Plasmodium malariae, P. vivax, P. falciparum, P. ovale

What is the diagnosis of malaria based on? The demonstration of the Plasmodium species in theblood.

What is based on agglutination of horse Monospot Test

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erythrocytes by heterophile antibody in infectious mononucleosis?How is the specific heterophile antibody distinguished from the Forssman type?

By differential absorption of the patient's serum

Serum or plasma is absorbed with? Both guinea pig kidney and beef erythrocyte stroma

What does Guinea pig kidney absorb? Only heterophile antibodies of the Forssman type

What does beef erythrocytes absorb? Only the heterophile antibody of infectious mononucleosis

A positive reaction for heterophile antibody occurs when?

Agglutination of horse RBCs by the absorbed patient specimen happens

Agglutination of horse RBCS by the absorbed patient specimen indicates____ for heterophile antibody

A positive reaction

Guinea pig kidney contains only the ___ and beef erythrocytes contain only the __

Forssman antigen, antigen associated with infectious mononucleosis

Whole blood is added to varying concentrations of sodium chloride solution and allowed to incubate at room temperature in the ____ test?

Osmotic fragility test

How is the amount of hemolysis determined? By examining the supernatant fluid either visually or with a spectrophotometer

The main factor in this procedure is? The shape of the erythrocyteThe shape of the erythrocyte depends on? The volume, surface area, functional state of the

erythrocytic membraneTrue or False: The fragility of erythrocytes is increased when the rate of hemolysis is increased

TRUE

True or False: If the rate of hemolysis is increased, the erythrocytic fragility is decreased

FALSE

Which test demonstrates the sensitivity of erythrocytes to the protein complement?

Sucrose Hemolysis Test

When do erythrocytes in PNH lyse? When exposed to serum solutions of low ionic strength containing complement

Which cells are tartrate resistant? Hairy cell leukemia, Sezary syndrome, some T-cell acute lymphoblastic leukemias

Most of the acid phosphatase isoenzyme is inhibited by?

L-tartaric acid

Where are peripheral blood or bone marrow smears fixed and incubated?

In a solution of naphthol As-BI phosphoric acid and fast garnet GBC salt

True or False: Lymphocytes display more activity than other leukocytes.

FALSE

Leukocyte alkaline phosphatase(LAP) activity is often used to distinguish?

Leukemoid reactions and chronic granulocytic (myelogenous) leukemia

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In a variety of conditions LAP activity can be ___?

Increased, normal, or decreased

What is the leukocyte alkaline phosphatase (LAP) procedure often used to distinguish?

Leukemoid reactions and chronic granulocytic (myelogenous) leukemia

Which best describes esterases? Ubiquitous in nature and encompass a variety of different enzymes acting on selective substrates

This method distinguishes what? Cells of the granulocytic series from cells of the monocytic series, useful in differentiation of leukemias

How are positive reacting cells differentiated from monocytes?

Sodium fluoride is incorporated with the incubation system.

In the alpha- naphthyl acetate esterase procedure, which is incubated with naphthol AS-D chloroacetate?

Blood or bone marrow smears

Naphthol compounds are coupled with what? Diazonium salt, forming highly colored deposits at the sites of enzyme activity

Which represent unstable types of hemoglobin, denatured by dyes and appear as intraerythrocytic stained bodies?

Heinz bodies

How are Heinz bodies formed? When the glycolytic enzymes in the erythrocytes are unable to prevent the oxidation of hemoglobin

What is the significance of increased numbers of Heinz bodies?

Unstable forms of hemoglobin in hemolytic disorders such as G6PD.

What happens shortly after birth to the amount of fetal hemoglobin (Hb F)?

It decreases to low levels

Increased amounts of Hb F are found in which conditions?

Sickle cell anemia, thalassemias, and hereditary persistence of fetal hemoglobin

What happens when leukocytes are treated with Periodic Acid–Schiff (PAS)?

When treated with periodic acid, glycols are oxidized to aldehydes. After reaction with Schiff’s reagent (a mixture of pararosaniline and sodium metabisulfite) a pararosaniline adduct is released that stains the glyco-containing cellular elements. Clinically, the PAS stain is helpful in recognizing some cases of erythroleukemia and acute lymphoblastic leukemia.

How is Myeloperoxidase (MP) detected? Myeloperoxidase (MP) is detected by means of the enzyme’s interaction with diaminobenzidine (DAB), a benzidine substitute. The brown reaction product is first intensified with copper salts followed by Gill’s modified Papanicolaou stain, which results in intense gray-black granules at sites of neutrophil and monocyte myeloperoxidase activity.This procedure differentiates cells of lymphoid origin from granulocytes and their precursors

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and monocytes.What is the Prussian blue reaction? The Prussian blue reaction precipitates free iron

into small blue or blue-green granules in erythrocytes. Free iron is not identifiable on Wright-stained or Wright-Giemsa–stained blood smears.

What is a sideroblast ? A sideroblast or siderocyte is an immature or mature erythrocyte containing free iron. Increased numbers of siderocytes are seen in disorders such as thalassemia major or in patients after a splenectomy.

When does a ring sideroblast occurs? If the iron granules encircle the nucleus of the erythrocyte, it is referred to as a ring sideroblast. Although alcoholism is the most common cause of ring sideroblasts, they may also be seen in cases of lead poisoning or anemia.

When is a Sudan Black B Stain performed? Following fixation, blood or bone marrow films are immersed in a buffered Sudan black B solution. After rinsing, slides are counterstained with Mayer hematoxylin. Cells are examined microscopically for the presence of blue-black discrete granulation.Cells committed to the lymphoid pathway display negative staining reactions, whereas myeloid and monocytoid forms display characteristic positive reactions. The Sudan black B staining pattern usually parallels the myeloperoxidase stain and is useful in the identification of myelogenous and myelomonocytic leukemias.

What is a Terminal Deoxynucleotidyl Transferase Test and where is it found?

Terminal deoxynucleotidyl transferase (TdT) is a non–template-directed DNA polymerase that catalyzes the irreversible addition of deoxynucleotides to the 3-hydroxy groups on the end of DNA. The primary methods of detection are immunofluorescence and immunoperoxidase using a monoclonal antibody.TdT is a cell marker found on immature and neoplastic cells frequently seen in leukemic states.

What is a Specimen quality procedure? All coagulation testing critically depends on the quality of the specimen. Minimum tissue trauma and the avoidance of hemolysis are essential. Proper phlebotomy techniques must be strictly followed.

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What is a Special collection techniques? To reduce the possibility of introducing tissue thromboplastin into a whole blood sample and the subsequent utilization of certain factors with clot formation, certain techniques should be followed.

What are some general sources of error? All procedural directions must be strictly followed because variations in pH, reagent concentration, and temperature are major sources of error.

How is quality control maintained? Water baths and heat blocks must be monitored continually for accurate temperatures.Refrigerators and freezers should also be monitored to ensure the stability of specimens and test reagents.Reagents must not be beyond their stated expiration date. Procedural technique must be consistent and appropriate.

List some of the Coagulation Procedures Activated partial thromboplastin time, Antithrombin III, Bleeding time, Circulating anticoagulants, D-dimer assay, Euglobulin lysis time, Substitution studies and factor assays,Fibrin split products, Fibrinogen assay

Describe the principle of the Activated Partial Thromboplastin Time procedure

The aPTT procedure measures the time required to generate thrombin and fibrin polymers via the intrinsic pathway. Although a partial thromboplastin time test can be performed, contact factors can be activated more thoroughly by the addition of substances such as kaolin in the activated form of this assay.In the aPTT, calcium ions and phospholipids that substitute for platelet phospholipids are added to blood plasma. The generation of fibrin is the end point.

How is the aPTT used? –Clinically, the aPTT is used to identify and quantitate deficiencies in the intrinsic clotting system and to control anticoagulant therapy.

How are the aPTT results reported? Reference values are dependent on the activator and phospholipid reagents used; however, 20 to 35 seconds is typically normal. In some laboratories, ranges may be from 28 to 42 seconds, with 42 to 46 seconds being marginal.

What are the Clinical applications of the aPTT?

The aPTT is widely advocated as the test of choice for the control of heparin therapy. It is also important in the screening profile of prekallikrein; high-molecular-weight kininogen;

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factors XII, XI, IX, VIII, X, V, II, and I; and inhibitors against these factors.

Describe the principle of the Antithrombin (AT III)

In the presence of heparin, thrombin is neutralized at a rate that is proportional to the antithrombin (AT III) concentration. Following defibrination, plasma is assayed in a two-stage procedure that utilizes standardized amounts of heparin, fibrinogen, and thrombin. The resulting clotting time is interpreted using a calibration curve.Clinically, the AT III assay is useful prior to and subsequent to treatment with heparin in cases of disseminated intravascular coagulation (DIC).

What is the advantage of the Bleeding Time test?

The bleeding time test is an in vivo measurement of platelet adhesion and aggregation on locally injured vascular subendothelium. This test provides an estimate of the integrity of the platelet plug and thereby measures the interaction between the capillaries and the platelets.Clinically, the bleeding time is prolonged in thrombocytopenia, qualitative platelet disorders such as von Willebrand disease, aspirin ingestion, or the presence of vascular problems

What is a circulating anticoagulant? When coagulation deficiencies are caused by inhibitors to specific factors rather than the lack of a factor, these inhibitors are sometimes referred to as circulating anticoagulants .

How can a circulating anticoagulant be detected?

To detect a circulating anticoagulant, the aPTT and the PT that were originally abnormal are repeated using various dilutions of patient plasma and normal plasma.The detection of an inhibitor may show up immediately or may require incubation of the normal plasma in the presence of the inhibitor. Differentiation between a coagulation factor deficiency and a circulating anticoagulant is important.

How can a circulating anticoagulant be reported?

If the abnormality is that of a deficiency, a normal plasma sample will correct the assay results to a reference value.If the abnormality is caused by a circulating anticoagulant (inhibitor), a greater correction is demonstrated as the ratio of normal plasma increases in the mixture.

What are some of the anticoagulants that may Various types of anticoagulants may interfere

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interfere with coagulation? with coagulation at different stages, especially factor VIII, heparin-like activity, and antithromboplastins. Most acquired anticoagulants are autoantibodies (usually IgG, sometimes IgM) directed against specific coagulation factors.

What is principle of the D-dimer assay? The fundamental principle of the D-dimer assay has remained largely unchanged: recognition of the unique neoepitope in D-dimer by specific antisera. D-dimer contains a neoepitope that is formed following the cross-linking of adjacent D domains by factor XIIIa. It is this epitope that is recognized by specific antisera used in clinical assays.

Explain the principle of the Euglobulin Lysis Time

The euglobulin fraction of plasma contains plasminogen, fibrinogen, and activators with the potential for transforming plasminogen to plasmin. This fraction is precipitated with 1% acetic acid and resuspended in a borate solution. The euglobulins are then clotted by the addition of thrombin. The clot is incubated and the time of lysis is reported. Clinically, the euglobulin lysis test is a screening procedure for fibrinolytic activity.

Describe Substitution studies Assay and how they can be performed

Substitution studies and factor assays can be used to identify specific coagulation factor deficiencies.Substitution studies may be performed using adsorbed plasma and aged serum with the aPTT to identify deficiencies of blood coagulation. Substitution studies may also be performed using adsorbed plasma with the PT to identify a factor VII deficiency.

What is the principle of Specific factor assays?

Factor assays are based on the ability of the plasma in question to correct a factor-deficient substrate, such as factor VIII. The actual assay is the same as the aPTT with the exception of the factor-deficient substrate. Identification of specific factor deficiencies is valuable in both the diagnosis and the treatment of patients.

When conducting a Fibrin Split Products (FSP) test, what is the purpose of adding thrombin as well as soy bean enzyme inhibitors to the whole blood sample?

Thrombin is added to ensure complete clotting of the sample, and the soy bean enzyme inhibitors are added to prevent any breakdown of fibrin.

What is demonstrated from FSP elevated results?

Elevated results demonstrate that the activation of plasmin has occurred or is occurring, such as

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in primary fibrinolysis and DIC with secondary fibrinolysis.

What is the principle behind a fibrinogen assay?

When plasma is diluted and clotted with excess thrombin, the fibrinogen concentration is inversely proportional to the clotting time

Fibrinogen assays are useful in detecting deficiencies of fibrinogen and in detecting an alteration in the conversion of fibrinogen to fibrin.

What is the use of the simple protein, protamine?

– Protamine is used to neutralize the effects of heparin.

What is the indication of a positive result from a Protamine Sulfate Assay?

– Inappropriate presence of intravascular fibrin monomers

What is being measured when conducting a prothrombin time?

– Length of time required to form a fibrin clot (measured in seconds)

What are some situations in which prothrombin time test is used?

to monitor oral anticoagulant therapy as a screening test in the diagnosis of coagulation deficiencies– as a component of a liver profile

assessmentWhat is indicated from prolonged results in a prothrombin time?

Deficiency of one or more factors in the extrinsic pathway: factors VII, X, and V and factor II or I. The presence of an inhibitor will also produce prolonged values.

–What is determined from a thrombin time test?

Determines the rate of thrombin-induced cleavage of fibrinogen to fibrin monomers and the subsequent polymerization of hydrogen-bonded fibrin polymers

What might cause the abnormal result of a thrombin time test?

Extremely low fibrinogen levels Abnormal fibrinogen thrombin inhibitors High concentrations of immunoglobulin

(e.g., myeloma proteins) Presence of heparin

High concentrations of fibrin-fibrinogen degradation products

How is cell size and quantity determined based on the use of an electrical impedance procedure?

Cell counting and sizing are based on the detection and measurement of changes in electrical impedance (resistance) produced by a particle as it passes through a small aperture.

Which method of cell counting uses a laser light?

Optical Detection method

How does the diluted blood specimen interact with the laser light during optical detection?

A diluted blood specimen passes in a steady stream through which a beam of laser light is focused.

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As each cell passes through the sensing zone of the flow cell, it scatters the focused light.Scattered light is detected by a photodetector and converted to an electrical pulse.

What is represented by the number of electrical pulses detected in an optical detection method?

The number of pulses generated is directly proportional to the number of cells passing through the sensing zone in a specific period.

What are the three operational processes of light amplification generated by emission of radiation?

Diffraction and the bending of light around corners with the use of small angles

Refraction and the bending of light because of a change in speed with the use of intermediate angles

Reflection and light rays turned back by the surface or an obstruction with the use of large angles

How is Radio Frequency (RF) used to detect cell size?

– High-voltage electromagnetic current is used to detect cell size, based on the cellular density.

Why is laser light the most common light source used in flow cytometers?

– Because of the properties of intensity, stability, and monochromatism.

What is flow cytometry? – The simultaneous measurement of multiple physical characteristics of a single cell, as the cell flows in suspension through a measuring device.

How can flow cytometry rapidly classify groups of cells?

– Flow cytometry combines the technologies of fluid dynamics, optics, lasers, computers, and fluorochrome-conjugated monoclonal antibodies that rapidly classify groups of cells with heterogeneous mixtures.

In flow cytometry, what intrinsic properties of a cell are detected?

– Forward- and right-angle light scatter, which correlate with size and granularity of a cell, respectively.

Regarding scattered light, what is a forward scatter?

– Light scattered along the axis of the laser beam

Regarding scattered light, what is a side/ orthogonal scatter?

– Light scattered perpendicular to the axis

Forward scatter is proportional to? – Cell sizeSide scatter is proportional to? – Cytoplasmic granularityDefine parameter: – Statistical term that refers to any

numerical value that describes an entire population.

Major types of automation are representations of what?

Representative of the ways that blood cells can be counted, leukocytes differentiated, and other components (e.g., MCH and MCHC) calculated

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How does hemoglobin being measured? Measured by the traditional cyanmethemoglobin flow-cell method at 525 and 546 nm, depending on the instrument manufacturer

What are histograms? Histograms are graphic representations of cell frequencies versus sizes.

In a homogeneous cell population, the curve assumes what shape or distribution?

A symmetrical bell-shaped or gaussian distribution.

In a histogram, a wide or more flattened curve is seen in what?

A wide or more flattened curve is seen when the standard deviation from the mean is increased.

True or false.Histograms not only provide information about erythrocyte, leukocyte, and platelet frequency and their distribution about the mean but also depict the presence of subpopulations.

True

True or FalseHistograms provide a means of comparing the sizes of a patient’s cells with normal populations.

True

True or False.In a histogram, shifts in one direction or the other can be of diagnostic importance.

True

Tue or FalseIn the Coulter system, the size (volume in femtoliters) is represented on the y-axis.

False (x-axis represents the size)

In analysis of instrumental data output includes what?

The erythrocyte histogramQuantitative descriptors of erythrocytesThe leukocyte histogramsPlatelet histogramsDerived platelet parameters

What does an erythrocyte histogram reflects? The erythrocyte histogram reflects the native size of erythrocytes or any other particles in the erythrocyte size range.

The erythrocyte histogram in the Coulter system displays cells as small as __(1)___fL., but only those greater than ___(2)___fL are counted as erythrocytes.

1) 24 fL2) 36 fL

In a erythrocyte histogram, what is the advantage of the extension of the lower end of the scale from 36 to 24 fL?

It allows for the detection of erythrocyte fragments, leukocyte fragments, and large platelets

An expression of erythrocyte size is the _______ in the Coulter series. This term refers to variation in erythrocyte size.

RDW

Correlations between the _______ and the _________exist for various types of anemia.

RDW and the MCV

What does size-referenced leukocyte The classification of leukocytes according to

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histograms displays? size following lysis. It does not display the native cell size

What does a platelet histogram reflects? Platelet counting and sizing in both the electrical impedance and the optical systems reflect the native cell size.

What is the measure of the average volume of platelets in a sample?

MPV

True or FalseThe MPV is analogous to the erythrocytic MCV.

True

True or FalseMean platelet volume calculation is derived from the same data as the platelet count.

True

True or FalseIn healthy patients, there is an inverse relationship between platelet count and size.

True

What are the three steps in a laser technology?

Cytochemical reactions prepare the blood cells for analysis.A cytometer measures specific cell properties.Algorithms convert these measurements into familiar results for cell classification, cell count, cell size, and hemoglobinization.

RBC/platelet channel uses a laser-based optical assembly that is shared with what channel?

The basophil/lobularity channel.

Where is the platelet histogram derived? The platelet histogram is derived from measurements made with the high-angle detector.

What is the mode of the measured platelet volumes?

MPV

Most flow-cell instruments can simultaneously analyze multiple parameters at what rate?

at the rate of 5,000 to 10,000 cells/sec.

In flow cytometry, the cellular analysis yields what properties of individual cells?

The cellular analysis yields quantitative data about the chemical and physical properties of individual cells.

Major advances in this technology are owing to several factors, what are these factors?

The ability to produce monoclonal antibodies resulted in the subsequent development of specific surface markers for various subpopulations of cells.

The development of new fluorescent probes for DNA, RNA, and other cellular components increased the variety of possible applications at the molecular and cellular levels.

The expansion of computer applications has

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improved the instrumentation technology, making it easier to operate and more practical for use in clinical as well as research laboratories.

In hematology, automated differentials can be based on a variety of principles which includes what?

– Determination of cell volume by electrical impedance or forward light scatter, cytochemistry or peroxidase staining, and VCS technology.

Because single-cell suspensions of peripheral blood and bone marrow are easy to obtain, most clinical applications of flow cytometry are in the specialties of __________ and ______________.

– hematology and immunology

Reticulocytes Enumeration by flow cytometry is more ____________.

– accurate, precise, and cost-effective.

Platelet counts can provide an estimate of young, reticulated platelets by counting platelets that stain with what kind of dye?

– with an RNA dye.

What kind of typing identified by a cluster designation (CD), are used in most flow cytometry immunophenotyping.

– Immunophenotyping monoclonal antibodies

A cellular application that has become an important tool in the diagnosis and classification of hematologic neoplasia by immunophenotyping. This techniques with bone marrow cells are applicable to DNA cell cycle analysis.What is this technique?

– Flow Cytometry

What kind of new technology that referred to as automated digital cell morphology, provides an unprecedented level of efficiency and consistency. In its simplest form, automated digital cell morphology is a process where blood cells are automatically located and preclassified into categories of blood cell?

– Digital Technology

This is the earliest instruments to detect blood clotting. This uses a methodology to measure the conduction or impedance of an electrical current by the formation of fibrin. What kind of instrumental methodology is this?

– Electromechanical methods

This principle/methodology measures is the change in light transmission as optical density (absorbance) versus time can be used to quantitatively determine the activity of various coagulation stages or factors.

– Photo-optical Method

_______ is defined as the resistance that a – Viscosity

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material has to a change in its form.This principle usesthe natural thickening (viscosity) and monitored by the motion (amplitude of an oscillating steel ball in a specially designed cuvette), as a change in form takes place.

– Viscosity-Based Detection System (VDS)

What type of platelet agglutination method measures the ability of a patient’s plasma to agglutinate formalin-fixed platelets in the presence of ristocetin?

– ristocetin cofactor assay

The principle of the test is that platelet-rich plasma is treated with a known aggregating agent. What is this test?

– Platelet Aggregation

A new type of automated system that incorporates a high shear flow system to simulate the in vivo hemodynamic conditions of platelet adhesion and aggregation as encountered at a vascular lesion.

– Siemens PFA-100

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