1.Hematological Disorders

8/7/2019 1.Hematological Disorders

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HEMATOLOGICAL DISORDERS

HEMATOLOGICAL DISORDERS

The blood and the blood forming sites, including the bone marrow and the

reticuloendothelial system

Blood

Plasma

Blood cell

Blood Cells

Erythrocyte: RBC

Leukocyte: WBC

Neutrophil

Eosinophil

Basophil

Monocyte

Lymphocyte:

T lymphocyte

B lymphocyte

Thrombocyte: platelet

ANEMIA

- Decrease availability of oxygen totissues due to insufficient RBC

Etiology:

Decrease production of healthy RBC

Increased RBC destruction

Loss of blood

Inadequate dietary intake of vitamins & minerals

Infections

Drugs and chemicals

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Types of Anemia according morphologic characteristics of RBC

1. Normocytic/Normochromic

Acute blood loss

Hemolysis

Chronic renal disease

Cancer

Diseases of endocrine dysfunction

Aplastic anemia

Pregnancy

2. Macrocytic/Normochromic

Vitamin B12 deficiency

Folic acid deficiency

Liver disorders

Alcoholism

Splenectomy

3. Microcytic/hypochromic

Fe deficiency anemia

Thalassemia

Lead poisoning

4. Abnormal shape of RBC

Sickle shape anemia

Hereditary spherocytosis

Manifestations

Pallor

Easy fatigability

Weakness

Weight loss and Anorexia

Shortness of breath

Headache/dizziness

Amenorrhea

Cold sensitivity

Tachycardia

Paresthesia

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Diagnostics

Peripheral blood smear

CBC and Fe profile

Decrease hgb (6 to 9g/dl)

Decrease serum Fe level

Increase total Fe-binding capacity

Absence of hemosiderin

Determination of source of blood loss

sigmoidoscopy, colonoscopy

Upper & lower GI series

Stool & urine occult blood exam

Management

Correction of chronic blood loss

Diet & supplemental Fe preparations

Oral FeSO4

Preferred & least expensive

E.g. Ferrous sulfate (Feosol)

Ferrous gluconate (Fergon)

Should be given p.c.

Use straw for oral liquid Fe

Take Vitamin C

Do not administer with milk, antacid

Parenteral Fe

Administered as deep IM or IV

Imferon (IM)

Use Z tract

Do not massage site of injection

Fe Dextran

Should be infuse not >1ml/min

Antidote: deferoxamine mesylate

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Indications for parenteral Fe

Malabsorption syndrome

Intolerance to oral iron

Persistent blood loss

Compliance of patient

Rapid response is needed

Supportive nursing care

- Promote rest

- Provide good oral and skin care

B. Folic Acid Deficiency Anemia

Megaloblastic anemia with slow and insidious onset

- Amount of folic acid is insufficient to synthesize DNA, RNA & proteins

Serum folate level: < 4mg/ml

Etiology and risk factors:

Inadequate dietary intake

Chronic alcoholism

Eating disorders

Long term use of anticonvulsants

Cancer & leukemic patients

Pregnancy

Use of certain oral contraceptives

Malabsorption condition

Clinical manifestations:

General s/sx of anemia

No neurological manifestations

Diagnostics:

BME and PBS confirmatory test

Schillings test

Management:

Therapeutic trial:

50-100mg of folic acid/IM daily x 10 days

Oral folic acid replacement

Nutritional support and proper food preparation

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Vitamin C is sometimes prescribed

C. Pernicious Anemia

Megaloblastic or macrocytic caused by failure of absorption of Vitamin B12 (cobalamin)

Autoimmune disorder characterized by absence of intrinsic factor in gastric secretions

Common between 40 -70 years old

Peak incidence: 70 years old

Prevalent in Celtic & Scandinavian ancestry

Pathophysiology

Intrinsic factor production (by the parietal cells of the stomach)

Vit B12 absorption

RBC production

DNA synthesis

Impairment of integrity of cells

Causes:

Insufficient dietary intake

Certain medications (neomycin, OCP)

Genetic disorders ( tanscobalamin II def.)

Gastric surgery

Clinical Manifestations:

- General s/sx of anemia

- GI manifestations

- neurologic disorders

Diagnostics:

CBC

PBS

Serum Fe profile

Gastric secretion analysis - to check for the presence of free HCL

Schilling test

Definitive test

Oral radioactive Vit B12 is adm.after parenteral infusion of intrinsic factor

Collect 24-hour urine specimen

(+) Result: excretion of Vit B12

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Management:

Cobalamin Therapy

Parenteral cobalamin standard treatment

Cyanocobalamin or hydroxycobalamin

1000ug daily for 2 weeks

Then weekly (10 weeks), then monthly tx for life

Fe and folic acid supplements

Monitor blood counts

D. Aplastic Anemia

- Hypoplasia of the bone marrow leading to pancytopenia

- Fat replaces bone marrow

- insidious or rapid onset

Causes:

Hereditary (Fanconi syndrome)

Acquired 80%

High dose of radiation and chemotx

Certain drugs

Autoimmune disorder

Infectious agents

Pregnancy

Idiopathic

Most common in adolescents and young adults

Clinical manifestations

General s/sx of anemia

Freq. infections

Unexplained bruising

Prolonged bleeding

Severe manifestations

Pancytopenia

Normocytic anemia

Neutropenia

Thrombocytopenia

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Diagnostic test

Bone marrow aspiration/biopsy

Adult: post. Iliac crest most common site

Prone position during the procedure

Child: tibia most common site

Side-lying position during the procedure

Management:

Withdrawal of offending agent

Blood transfusion

- Mainstay therapy if 2 to myelotoxic agents

Treatment of infection

Immunosuppressive therapy (Antithymocyte Globulin- ATG)

Bone marrow transplant

- Tx of choice for production

Causes:

Hereditary (G6-PD enzyme def., sickle cell anemia, thallasemia)

Acquired

- Exposure to toxic chemicals, drugs

- Trauma

- Infections & immune reactions

Clinical manifestations: general

Diagnostics:

1. CBC

2. Blood smear

3. RBC fragility

4. Shortened RBC lifespan

5. Fecal and urinary urobilinogen

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Management:

Withdrawal of offending agent

Transfusion therapy

Folic acid & Fe supplement

Splenectomy

- tx of choice for hereditary

Erythropoietin injection

B. Sickle Cell Anemia

-autosomal recessive disorder affecting hemoglobin

Etiology & Risk factors:

Prevalent in areas where malaria is endemic

Children are rarely symptomatic until late 1st year (r/t increase amt of fetal hgb (HgbF)

Classification:

Sickle cell anemia: homozygous for sickle cell gene

Sickle cell trait:

- Heterozygous

- Milder form

- Carrier state of HbS

- 8% of African Americans are carriers

- Malaria resistant


1. Vasoocclusive crisis

- aka pain crisis

- Most common & non-life-threatening

- Results from sickled cells obstructing blood vessels, causing occlusion, ischemia

&potential necrosis

S/sx:

Fever

Acute abdominal pain

Hand- foot syndrome (dactylitis)

Priapism

Arthralgia

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2. Sequestration crisis

- Results from sudden & massive trapping of destroyed RBC by visceral organs especially

spleen

- Most commonly occurs between 8 months & 5 years old

- Death is due to anemia or cardiovascular collapse

- Functional asplenia = termed for chronic manifestation

3. Aplastic crisis

4. Hyperhemolytic crisis

5. Acute chest syndrome

- Most common cause of mortality

- Char. by chest pain, fever, cough, tachypnea, pulmonary infiltrates

- Fat emboli major etiology

6. CNS involvement

Most prevalent in childhood & adolescence

Stroke (thrombotic) most severe symptom

7. Overwhelming infection

Streptococcus pneumoniae

Haemophilus influenza type B

8. Chronic symptoms

Jaundice

Gallstones

Delayed sexual maturity

Growth retardation

Diagnostics:

Blood smear

Sickle turbidity tube test

For mass screening of HbS

Hgb electrophoresis diagnostic test

- Use to diff. sickle cell disease to SC trait

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Management:

Prevention of sickling phenomenon

- Adequate oxygenation

- Adequate hydration

- Administration of hydroxyurea to fetal hgb

- Monthly blood transfusion

Treatment of crisis

- Hydration/electrolyte replacement

- Antibiotic therapy

- Pain management: rest

- Blood products

Genetic counseling

C. Polycythemia Vera

Hyperplasia of bone marrow

Erythrocytosis, leucocytosis,thrombocytosis

Caused by unregulated neoplastic proliferation

Peak incidence: 50-70 y/o with Jewish descent

Pathophysiology:


Ruddy complexion

Cardiovascular HPN (dizziness, headache)

Fatigue

Shortness of breath

Increased clotting leading to stroke & MI

Hepatosplenomegaly

Skeletal gout

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Diagnostics:

CBC

RBC count - inc. (8-12 M)

Hgb, hct level inc.(18-25g/dl)

Platelet count

Bone marrow biopsy

ABG

Serum uric acid level determination

Management:

No permanent cure

Increase fluid intake

Monitor s/sx of bleeding & thromboembolism

Administer analgesic & antihistamine as ordered

Therapeutic phlebotomy

Chemotherapy

Radiation therapy (Na phosphate/IV)

D. Hemochromatosis (HH)

- aka iron overload disease

- Inherited metabolic disorder that causes increased absorption of Fe that is deposited in

the body tissues, organs

- HH seldom manifest until adulthood

- Defective gene: HFE

Risk factors: M=F (Males dev. symptoms at younger age)


Joint pain most common

Depression

Bronzing of the skin

Fatigue

Impotence

Arthritis

Loss of body hair

Liver, pancreas, heart problem

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Diagnostics:

- Transferring saturation test (TS)

- Serum ferritin test

- HFE mutation determination - definitive test

Management:

Phlebotomy

Avoid alcoholic beverages

Limit dietary intake of food rich in Fe

E. Thalassemia Major ( Cooleys Anemia)

- Autosomal recessive disorder

- Basic defects: Deficiency in the synthesis of beta chain polypeptides

- Mediterranean, Africa, SouthEast Asian origin

Classification:

1. Alpha Thalassemia

Most common

Heterozygous

Benign & asymptomatic

2. Beta Thalassemia minor

Mild to moderate Microcytic anemia

Rate of production of globin molecule

Insidious onset

3.BetaThalassemiamajor

Homozygous

Incompatible with life

Severe microcytic ,hypochromic anemia

Clinical manifestations: (s/sx noted toward the end of the 1st year)

Severe anemia

Unexplained fever; headache

Anorexia, poor feeding

Enlarged abdomen; splenomegaly, hepatomegaly

Impaired physical growth

Listlessness; exercise intolerance

Failure to thrive

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Diagnostics:

Amniocentesis

Peripheral blood smear

Hgb electrophoresis

Management:

Chronic blood transfusion

Iron chelation with deferoxamine

Splenectomy

Bone marrow transplantation

Dietary intervention

- Vitamin C intake

- Increase tea consumption

Genetic counseling

DISORDERS OF PLATELETS AND CLOTTING FACTORS

Idiopathic Thrombocytopenic Purpura

- Most common thrombocytopenic disorder

- Hemorrhagic autoimmune disease that results in platelet destruction upon reaching liver &

spleen


Petechiae

epistaxis

Ecchymosis

Easy bruising

Bleeding gums

Diagnostics:

Bone marrow aspirate megakaryocytes

Platelet count < 100,000/mm3

Prolonged bleeding time

Normal coagulation time

Increased capillary permeability

(+) platelet Ab screening

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Complications:

Spontaneous cerebral hemorrhage

Severe nose, GIT and urinary bleeding

Bleeding into the diaphragm

Nerve pain or paralysis

Management:

Administer high dose corticosteroids

Plasmapheresis - short term therapy

Surgery

IV gamma globulin

Splenectomy

Immunosuppressive therapy

(eg. Vincristine, cyclophosphamide)

COAGULATION DISORDERS

A. Hemophilia

Defect in clotting mechanism of the blood

Genetic disorder; X-linked recessive transmission

Usually occurs in males

Females carriers

Classification:

1. Hemophilia Aclassic hemophilia

Factor VIII (antihemophilic globulin) deficiency

Lab Fx: prolonged coagulation time

Normal bleeding time

2. Hemophilia B Christmas disease

Factor IX def. ( plasma thromboplastin component)

Lab fx: similar with hemophilia A

3. Von Willebrands disease

Factor VIII deficiency & defective platelet dysfunction

Most common congenital bleeding disorder

Autosomal dominant

Lab fx: prolonged coagulation & bleeding time

Low factor VIII levels

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Decrease platelet adhesiveness


Prolonged bleeding

Hemarthrosis hallmark

Intracranial hemorrhage

Recurrent hematoma formation

Severity of Bleeding:

Mild bleeding with severe trauma or surgery

Moderate bleeding with mild to mod. Trauma

Severe spontaneous bleeding without trauma

Diagnostics:

1. Platelet count

2. Bleeding time

3. PT

4. PTT prolonged

Management:

1. Control of bleeding

2. Prevention of bleeding with use of factor replacement

Drugs that replace deficient coagulation factors:

Factor VIII concentrate from recombinant DNA

Factor IX concentrate

Adjunctive measures

DDAVP (deamino-D-arginine vasopressin)

- tx of choice for mild hemophilia & Von Willebrand

NSAIDs - avoid use of aspirin

Corticosteroids

Amicar (aminocaproic acid)

- Oral or local application

- Prevents clot destruction

Regular program of exercise and physical therapy

B. Disseminated Intravascular Coagulation

Bodys response to overstimulation of clotting and anti-clotting processes in response to

injury

Loss of balance between the clotting and lysing systems in the body

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Excessive thrombosis & excessive lysis occur simultaneously

Causes:

1. Infection

2. Introduction of tissue coagulation factors into the circulation

3. Damage to vascular endothelium

4. Hemolytic transfusion reaction

5. Obstetric complications ( abruption placenta, IUFD)

Pathophysiology:


Restlessness

Anxiety

Dyspnea

Coolness of extremities

Acute renal failure

Altered mental status

Signs of abnormal bleeding

Diagnostic findings:

Low fibrinogen

Prolonged PT

Pronged PTT

Reduced platelets

Elevated fibrin split products

Management:

Treat the underlying cause

Replacement therapy for serious hemorrhagic complications

- Fresh frozen plasma

- Platelet transfusion

- Cryoprecipitate

Supportive measures

Heparin (controversial)

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Prevent injury

Leukemia

Hematopoietic malignancy with unregulated proliferation of leukocytes

Types:

1. Acute myeloid leukemia

2. Chronic myeloid leukemia

3. Acute lymphocytic leukemia

4. Chronic lymphocytic leukemia

A. Acute Myeloid Leukemia (AML)

Defect in the stem cells that differentiate into all myeloid cells:

a. Monocytes, granulocytes, erythrocytes, and platelets

- Most common nonlymphocytic leukemia

- Affects all ages

b. Peak incidence at age 60

- Prognosis is variable

Manifestations:

Fever and infection

Weakness and fatigue

Bleeding tendencies

Pain from enlarged liver or spleen

Hyperplasia of gums

Bone pain

Treatment:

- Aggressive chemotherapy

- Induction therapy

- BMT

B. Chronic Myeloid Leukemia (CML)

Mutation in myeloid stem cell with uncontrolled proliferation of cells: Philadelphia

chromosome

Stages

A. Chronic phase

B. Transformational phase

C. Blast crisis

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CML

Uncommon in people under 20

Incidence increases with age;

Mean age is 55 to 60 years

Life expectancy is 3 to 5 years

Manifestations: (initially may be asymptomatic):

Malaise

Anorexia

Weight loss

Confusion

Shortness of breath due to leukostasis

Enlarged, tender spleen

Enlarged liver

Treatment:

Imatinib mesylate (Gleevec)

- Blocks signals in leukemic cells that express BCR-ABL protein

Chemotherapy, BMT, and PBSCT

C. Acute Lymphocytic Leukemia

Uncontrolled proliferation of immature cells from lymphoid stem cell

Most common in young children, boys more often than girls

Prognosis is good for children

- 80% event-free after 5 years,

- Survival drops with increased age

Manifestations:

Leukemic cell infiltration is more common with this leukemia

Symptoms of meningeal involvement

Liver, spleen, and bone marrow pain

Treatment:

Chemotherapy,

Imatinib mesylate if (+) Philadelphia chromosome

BMT and PBSCT

Monoclonal antibody therapy

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D. Chronic Lymphocytic Leukemia

Malignant B lymphocytes, most of which are mature, may escape apoptosis, resulting inexcessive accumulation of cells

Most common form of leukemia

More common in older adults and affects men more often

Survival varies from 2 to 14 years depending upon stage

Manifestations:

Lymphadenopathy

Hepatosplenomegaly

Fever and weight loss

Anemia & thrombocytopenia

Treatment: early stage may require no treatment,

Chemotherapy

Monoclonal antibody therapy

Laboratory tests

Leukocyte count, ANC, hematocrit, platelets, electrolytes, and cultures reports

Nursing Diagnosis of the Patient With Leukemia

Risk for bleeding

Risk for impaired skin integrity

Impaired gas exchange

Impaired mucous membrane

Imbalanced nutrition

Acute pain

Hyperthermia

Fatigue and activity intolerance

Impaired physical mobility

Risk for excess fluid volume

Diarrhea

Risk for deficient fluid volume

Self-care deficit

Anxiety

Disturbed body image

Potential for spiritual distress

Grieving diagnoses

Deficient knowledge

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Potential Complications:

Infection

Bleeding

Renal dysfunction

Tumor lysis syndrome

Nutritional depletion

Mucositis

Depression

Planning the Care of the Patient With Leukemia

Major goals include

- Absence of complications

- Attainment and maintenance of adequate nutrition

- Activity tolerance

- Ability for self-care

- To cope with the diagnosis and prognosis,

- Positive body image

- An understanding of the disease process and its treatment

Interventions

1. Mucositis

Frequent, gentle oral hygiene

Soft toothbrush, or if counts are low, sponge-tipped applicators

Rinse only with NS, NS and baking soda, or prescribed solutions

Perineal and rectal care

2. Improving Nutrition

- Provide oral care before and after meals

- Administer analgesics before meals

- Provide appropriate treatment of nausea

- Provide small, frequent feedings with soft foods that are moderate in temperature

- Provide a low-microbial diet

- Provide nutritional supplements

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Lymphoma

Neoplasm of lymph origin

Hodgkins lymphoma

Non-Hodgkins lymphoma

Hodgkins Disease

Unicentric origin

ReedSternberg cell

Suspected viral etiology

Familial pattern

Incidence occurs in early 20s and again after age 50

Excellent cure rate with treatment

Manifestations:

1. Painless lymph node enlargement

2. Pruritus

3. Fever

4. Sweats

5. Weight loss

Treatment: Determined by stage of the disease

Chemotherapy

Radiation therapy

Non-Hodgkin's Lymphoma (NHL)

Lymphoid tissues become infiltrated with malignant cells that spread unpredictably;

localized disease is rare

Incidence increases with age - the average age of onset is 50 to 60

Prognosis varies with the type of NHL

Treatment: determined by type and stage of disease

Interferon

Chemotherapy

Radiation therapy

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Multiple Myeloma

Malignant disease of plasma cells in the bone marrow with destruction of bone

M protein and Bence-Jones protein

Median survival is 3 to 5 years; there is no cure

Manifestations:

1. Bone pain

2. Osteoporosis

3. Fractures

4. Elevated serum CHON

5. hypocalcemia

6. Renal damage

7. Renal failure

8. Symptoms of anemia

9. Fatigue

10. Weakness

11. Increased serum viscosity

12. Increased risk for bleeding and infection

Treatment:

Chemotherapy

Corticosteroids

Radiation therapy

Biphosphonates

Prepared by:

NCM104 Instructors

HAUCON

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1.Hematological Disorders

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