Disorder of Clotting Factor
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DISORDER OF CLOTTING FACTOR
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DARAHKOMPOSISI :
SEL DARAH MERAHSEL DARAH PUTIHTROMBOSITPLASMA
FAKTOR PEMBEKUAN PROTEIN LAINNYA
FUNGSITRANSPORTASI
NUTRISI (PROTEIN, KH, LEMAK, MINERAL, VIT)
O2 DAN CO2
MEMPERTAHANKAN TEKANAN DARAH
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DARAH
DALAM KEADAAN NORMAL DARAH NORMAL, BENTUK CAIR DAN BERADA DALAM SISTEM SIRKULASI (P.DARAH)
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FAKTOR YG BERPERAN DLM TROMBOSIS-HEMOSTASIS1. P.DARAH2. FAKTOR KOAGULASI 3. TROMBOSIT4. ANTIKOAGULAN5. SISTEM FIBRINOLISIS
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Trombosis : Dikenalkan Virchow (abad 19) “ Triad of Virchow “ 3 faktor berperan : 1. Pembuluh darah tidak normal. 2. Perubahan aliran darah / stasis. 3.Perubahan komposisi darah ( hiperkoagulabilitas )
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HEMOSTASIS, (Virchow’s Triad)
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Function of HEMOSTASIS
ARREST BLEEDINGMAINTAIN BLOOD IN FLUID STATE
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HOMEOSTATIC HEMOSTASIS
Physiologic function Maintain blood in fluid state
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Hemostatic Balance
ATIII
Clotting Factors
Tissue factor*
PAI-1
AntiplasminTFPI
Prot. C
Prot. S
ProcoagulantProcoagulant AnticoagulantAnticoagulant
Fibrinolytic System
Hemostasis an equilibrium of physiological activators and inhibitors of coagulation
The equilibrium is fragile and several causes put patient at risk
The equilibrium is fragile and several causes put patient at risk
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HEMOSTASISPrimary Hemostasis
Blood vessel contractionPlatelet Plug Formation
Secondary HemostasisActivation of Clotting Cascade Deposition & Stabilization of Fibrin
Tertiary HemostasisDissolution of Fibrin ClotDependent on Plasminogen Activation
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HemostasisBV Injury
PlateletPlateletAggregation
Blood VesselBlood Vessel Constriction
CoagulationCoagulation Cascade
Stable Hemostatic Plug
Fibrin formation
Reduced
Blood flow
Tissue Factor
Primary hemostatic plug
Neural
Lab Tests•CBC-Plt•BT,(CT)•PT•PTT
Plt StudyMorphologyFunctionAntibody
Platelet Adhesion
and Activation
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Clinical Manifestation of Hemostatic defect
PtechiaPurpuraEchymosisHematomaHematemesisMelenaHemathrosisHemoptysisHematuriaEpistaxisGum bleeding
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Petechia : a minute, rounded spot of haemorrhage on a surface, such as skin, mucous membrane, serious membranePurpura : a condition in which haemorrhage occur in the skinEcchymosis: extravasasion of blood into the subcutaneous tissue. It is marked by purple discoloration of the skin, the color gradually changing to brown green and yellowHematemesis : the vomiting bloodHemoptysis : the spitting of blood from larynx ,pharynx, trachea, bronchi or lung
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Hematoma : a focalized extravasation of blood which soon clot to form a solid mass and readily became encapsulated by connective tissueMelena : the discharge of stool colored black by altered bloodHemathrosis : extravasation of blood into a jointHematuria : the discharge of urine containing bloodEpistaxis : bleeding from noseHematoschezia : the discharge of stool colored red or brown
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Disorders of HemostasisVascular disorders –
Scurvy, easy bruising, Henoch-Schonlein purpura.
Platelet disordersQuantitative - ThrombocytopeniaQualitative - Platelet function disorders – Glanzmans
Coagulation disordersCongenital - Haemophilia (A, B), Von-WillebrandsAcquired - Vitamin-K deficiency, Liver disease
Mixed/Consumption: DIC
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VASCULAR
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VascularPurpura, echymosisConnective tissue Ehler-Danlos SyndromeAging process senile purpura (Bateman’s disease)Infectious Meningococcus Rocky Mountain’s syndrometyphoid fever Roseola spotVit C deficiency scurvyImmunologic Henoch-Schonlein purpura
↑ fragility capillary infectious, vasculitis
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Senile PurpuraSenile Purpura
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Petechiae in Petechiae in VasculitisVasculitis
((RockyRocky MountainMountain SpottedSpotted FeverFever))
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Henoch-Schonlein purpura
20y Male, fever, painful symmetric polyarthritis for a day. During the next two days, edema and palpable purpura developed.
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Henoch-Schonlein purpuraImmune disorder
Children
Follows infection
Petechiae with edema and itching.
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VascularNon palpable purpura senile purpura scurvy use corticosteroidPalpable purpura Henoch-Schonlein syndrome
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THE ROLE OF PLATELET IN THE ROLE OF PLATELET IN HEMOSTASISHEMOSTASIS
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THE ROLE OF PLATELET IN HEMOSTASISTHE ROLE OF PLATELET IN HEMOSTASIS
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Platelet dysfunction:
Inherited Disorders: Bernard-Soulier disease
large platelets, failure of adhesion
Glanzmann’s thrombasthenia normal size, failure of aggregation
Acquired Disorders:Drugs : Aspirin AlcoholUremia,
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Platelet dysfunction
Quantitatif : - thrombocytemia - ITP - aplastic anemia - DHF - acute leukemia - hypersplenism
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Platelet Disorders - Features:
Mucocutaneous bleedingPetechiae, Purpura, Ecchymosis.Spontaneous bleeding after traumaCNS bleeding (severe, Plt)Prolonged bleeding time (BT)
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Dengue Hemorrhagic fever
Platelet deficiency..
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Petechiae
Do not blanch with pressure (cf. angiomas)Not palpable (cf. vasculitis)
(typical of platelet disorders)
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Idiopathic Thrombocytopenic Purpura (ITP)
Acute - children (post infection)Chronic - adults ( females, 20-40 yrs)autoimmune disorder antiplatelet antibodies (IgG)IgG coated platelets removed by spleenUsually megakaryocytes in BM
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COAGULATION DISORDERS
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Coagulation DisordersCoagulation Disorders
Heparin is a cofactor that allows antithrombin III to inactivate thrombin and Factor Xa
Thrombomodulin binds to thrombin, making it an anticoagulant whichthen activates anti-coagulant protein C.Protein C cleave factors Va and VIIIa
Hemophilia B (ChristmasDisease) results from deficiency of factor IX
Hemophilia A (classic)is due to reduced amount or reduced activity of Factor VIII
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Coagulation disorders:
Deficiencies of Clotting factorsOnset - delayed after traumaDeep bleeding
Into joints - Hemarthroses Into deep tissues – Hematoma large skin bleed – Ecchymoses
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Ecchymoses(typical of
coagulation factor disorders)
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Coagulation Disorders
Laboratory findings:
Normal bleeding time & Platelet count
Prolonged prothrombin time (PT)
deficiencies of II, V, VII, X
Prolonged thrombin time (aPTT)
all factors except VII, XIII
Mixing studies - normal plasma corrects PT or aPTT
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HEMOFILIA
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How do you get it?Hemophilia is a genetic disease and is passed on by the X chromosome (the chromosome that carries the clotting factor).If a boy gets the X chromosome that carries the hemophilia gene he will become a hemophiliac.If a girl get the gene, she will become the carrier of the gene, not showing symptoms of the disease though she may have a long or heavy menstrual cycle. The carrier has a 50% chance of passing the gene on to her children every time she gets pregnant.
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How do you get it ctd.
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– This is a diagram of the joints most commonly affected by Hemophilia. It most often occurs at the knees, hips, ankles, shoulders, and elbows
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– The most common muscles that bleed with Hemophilia are those in the the upper arm, upper leg (front and back), the calf and the front of the groin
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Classification
% normal factor level
Causes of bleeding
Severe < 1% bleeding after trivial injury or spontaneous
Moderate
1 - 5% bleeding after minor injury; occasional spontaneous bleeds
Mild 6 - 30 % following major trauma, surgical or dental procedures
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Factor VIII DeficiencyClassic hemophilia (hemophilia A):
X-linked disorder (affects 1º males)Most common - severe bleedingNormal : 50 – 150%Def mild = 5-30%; moderate = 2-5%, severe = <1% Abnormal aPTT – Intrinsic path.Diagnosis - factor VIII assayTreatment - factor VIII concentrateCryoprecipitate (less desirable)Concentrat factor VIII KoateRecombinat factor VIII
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PATOGENESE
Faktor VIII adalah suatu glikoprotein yang mengaselerasi kompleks pembekuan darah. Pematangan faktor VIII disintesa sebagai single chain polypeptide yang mengandung 2332 residu asam amino dan terdiri dari tiga rantai yang tersusun A1-A2-B-A3-C1-C2.
Sebagai hasil proses proteolitik, dibentuk oleh suatu heavy chain (HCh) yang dibentuk dari A1, A2, dan B sedangkan light chain (LCh) dibentuk dari A3, C1, dan C2.
Faktor VIII disebut juga dengan anti hemophilic factor (AHF) merupakan ko faktor yang efektif untuk membentuk faktor IXa. Dimana faktor VIIIa dan faktor IXa bersama dengan faktor trombosit yang sudah aktif membentuk suatu kompleks yang disebut functional factor X activating compleks.
Adanya faktor VIIIa kecepatan aktifasi faktor X oleh faktor IXa akan meningkat secara drastis
Pada penderita hemofilia pembentukan pembekuan darah tertunda sehingga pembentukan trombin juga tertunda
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Factor IX Deficiency
Christmas disease (Hemophilia B):
X-linked recessive disorder Indistinguishable from classic hemophilia (F VIII)Requires evaluation of factor VIII and IX activity levels to diagnoseNormal : 50 – 150%Def mild = 5-30%; moderate = 2-5%, severe = <1%Treatment - factor IX concentrateCryoprecipitate if factor IX unavailable
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Laboratorium
Pemeriksaan aktivitas faktor VIII dan IX, pemeriksaan koagulasi menunjukkan aPTT yang memanjang dan PT normal, bentuk ini juga dapat disebabkan oleh pemberian heparin atau adanya lupus antikoagulan. Adanya heparin dapat diekslude dengan pengobatan Hepzyne (suatu inaktive heparin) dan kemudian pemeriksan aPTT ulangan atau trombin time.
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PENATALAKSANAAN
Prinsip umum penatalaksanaan pasien ini adalah pencegahan terjadinya
perdarahan yaitu dengan menghindari trauma dan hindari penggunaan
obat-obatan yang mempengaruhi agregasi trombosit (aspirin) dan non
steroid anti inflamasi serta penyuntikan I.m
Berbagai produk darah telah digunakan untuk menjamin hemostasis;
Porcin FVIII, FEIBA dan konsentrasi aktivasi protrombin kompleks lain
telah digunakan beberapa tahun yang lalu dengan sukses.
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Other Medical Treatment Analgesics (no aspirin) Anti-inflammatory medications Good dental care Education – life long management Psychological counseling Acute and long term management of
musculoskeletal problems
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Von-Willebrand Disease:
Coagulation + PLT disorder:
Congenital disorderDeficiency of vWF molecule Part of FVIII, Mediates platelet adhesionProlonged Bleeding timeLow Factor VIII & long aPTTMucocutaneous bleeding
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Von-Willebrand Disease:
vWF: F-VIII & Plt function.Defective Platelet AdhesionSkin Bleeding Prolonged Bleeding time.Low Factor VIII levels.
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VON WILLEBRAND DISEASESPONTANEOUS BLEEDING FROM MUCOUS MEMBRANESEXCESSIVE BLEEDING FROM WOUNDSMENORRHAGIAOFTEN PROLONGED BLEEDING TIME WITH NORMAL PLATELET COUNT
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VON WILLEBRAND DISEASEAUTOSOMAL DOMINANT, USUALLYRARELY AUTOSOMAL RECESSIVEOFTEN MILDDIAGNOSIS MAY BE DIFFICULT AND REQUIRE SOPHISTICATED TESTS.POSSIBLY IS THE MOST COMMON INHERITED BLEEDING DISORDER.
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VON WILLEBRAND DISEASETYPE I IS MOST COMMON.
REDUCED QUANTITY OF CIRCULATING VON WILLEBRAND FACTOR (vWF)SECONDARY DECREASE IN FACTOR VIII BECAUSE vWF STABILIZES FACTOR VIIIAPPROXIMATELY 70% OF ALL CASES OF VON WILLEBRAND DISEASERELATIVELY MILD
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VON WILLEBRAND DISEASETYPE II HAS SEVERAL SUBTYPES, ALL WITH SELECTIVE LOSS OF HIGH MOLECULAR WEIGHT MULTIMERS OF vWF. (Functional deficiency)
IIA: HIGH MOLECULAR WEIGHT MULTIMERS ARE NOT SYNTHESIZED.IIB: ABNORMAL HIGH MOLECULAR WEIGHT MULTIMERS ARE SYNTHESIZED.
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TYPE II VON WILLEBRANDDISEASE, CONTINUED
TYPE IIB HIGH-MOLECULAR-WEIGHT MULTIMERS ARE RAPIDLY REMOVED FROM CIRCULATION.HIGH-MOLECULAR-WEIGHT MULTIMERS MAY CAUSE SPONTANEOUS PLATELET AGGREGATION (SIMILAR TO TTP).IN TYPE IIB MAY SEE MILD CHRONIC THROMBOCYTOPENIA LIKELY CAUSED BY PLATELET CONSUMPTION.
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VON WILLEBRAND DISEASECLINICAL/LAB FEATURES
PROLONGED BLEEDING TIMEUSUALLY NORMAL PLATELET COUNTPLASMA VWF LEVELS ARE REDUCED.SECONDARY DECREASE IN VIII LEVELSJOINT BLEEDS ARE SEEN ONLY IN SEVERE CASES.
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1. Gambaran klinis2. Pemeriksaan Laboratorium
Pemeriksaan Laboratorium Kombinasi hasil px Pemanjangan bleeding time (BT) Penurunan kadar FVW plasma Penurunan aktivitas F VIII Penurunan kadar kofaktor ristosetin
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Penanganan segeraPenanganan jangka panjang
Penanganan segera :Stop obat yg menghambat fungsi trombositPemberian FVWTransfusi tombosit.
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Penanganan Jangka Panjang:Hindari pemakaian obat yang memperberat kelainan trombosit (aspirin)Pemakaian kartu identitas penderita atau gelang peringatan.DDAVP (desmopresin)FVWKriopresipitatObat lain : Imunoglobuli intravena, premarine, Epsilon aminocaproic acid (EACA), Estrogen.
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Analog sintetik hormon antidiuretik, vasopresin.Pemberian IntravenaMeransang pengeluaran FVW dari sel endotel, agar FVW & F VIII cepat ↑ dlm plasma.Dapat diberikan untuk pasien PVW tipe 1 & 2ADosis 0,3 ug/kg BB, Pengenceran dgn 20-30 cc saline, IV selama 10-20 menit.
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Melalui transfusi plasma segar atau konsentrat plasma yg mengandung komplek FVW-F VIII.Kriopresipitat;
Dapat segera memperpendek BT, namun bertahan relatif singkat (6-12 jam).
Sediaan konsentrat F VIII/FVW: Humate P, Alphanate.
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Imunoglobulin Intravena:Dosis 1 gr/kg/ hari selama 2-3 hari, dapat ↓ kadar antibodi anti FVW.
EACAInhibitor fibrinolitikMencegah perdarahan pada pembedahan minor.Dosis 3-4 gr tiap 4-6 jam IV atau per oral, dimulai sesaat sebelum prosedur & dilanjutkan 5-7 hari.
Estrogen↑ produksi FVW oleh endotelKehamilan normal sering kadar FVW & F VIII normal.
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Secondary Hemostatic Disorders
Acquired coagulation disorder:
Vitamin K deficiency- neonates - decreased intestinal flora and dietary intake- oral anticoagulants (coumadin)- fat malabsorption syndromes
Required for factors II, VII, IX, X Prolonged PT and aPTT
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Combined Primary and Secondary Hemostatic Disorders
Severe Liver Disease: Primary - dysfunctional platelets and/or thrombocytopenia (
BT) Secondary - decrease in all coagulation factors except vWF (
PT, aPTT) Vitamin K will promote synthesis of factors II, VII, IX, X
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Clinical Features of Bleeding Disorders
Platelet Coagulation disorders factor disorders
Skin Deep in soft tissuesSite of bleeding Mucous membranes (joints, muscles) (epistaxis, gum, vaginal, GI tract)
Petechiae Yes No
Ecchymoses (“bruises”) Small, superficial Large, deep
Hemarthrosis / muscle bleeding Extremely rare Common
Bleeding after cuts & scratches Yes No
Bleeding after surgery or trauma Immediate, Delayed (1-2 days), usually mild often severe
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Platelet Coagulation
Petechiae, Purpura Hematoma, Joint bl.
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TROMBOSISDEFINISI : BEKUAN DI DALAM P.DARAH YG TERDIRI KOMPOSISI DARAH, FAKTOR PEMBEKUAN, SDM, DAN TROMBUSADA 2 JENIS : -TROMBUS MERAH
-TROMBUS PUTIH PENYEBAB KEMATIAN NO.1
DI AMERIKA SERIKAT / NEGARA BARAT
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PATOGENESISFAKTOR YG MENSTIMULASI TROMBOSISFAKTOR YG MENCEGAH TROMBOSIS
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FAKTOR YG MENSTIMULASI TROMBOSIS
ENDOTEL YG RUSAKAKTIFASI KOAGULASIAKTIFASI TROMBOSITSISTEM FIBRINOLISIS MENURUN
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COA GULATION, INHIBITOR AN D FIBRINOLYSISCOA GULATION, INHIBITOR AN D FIBRINOLYSIS
C a**
H M W K(F itz ge rald)
X II Ia
X III
HM W K
K al ikre in
X II
F ibrin ope ptid a A
F ibrin ope ptid a B
Fibri n M onomer Cross -l inke dFibr in
Fibrin Polymer
F ibrino gen I
P rekal ikrein(F let che r)
XI Ia
X I X Ia
IX IX a
P ro thrombin II Thrombin
V II IaV III
V V a
X aX
V II
VI Ia + TFHM W K
C a**
C a**
D-Dimer
C a**P L
P L
C a**
activate dProt C
Protei n C
Protein S
TM
Anticoagulant
ExtrinsicIntrinsic
Aktivatio n
Inhib itio n
Hepar in
Plasmin
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FAKTOR YG MENCEGAH TROMBOSIS
ENDOTEL UTUHANTIKOAGULAN ALAMIAH
AT IIIPROT CPROT SALPHA 1ANTITRIPSINALPHA 2 MAKROGLOBULIN
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MANIFESTASI KLINIS
STROKEINFARK MIOKARDTROMBOSIS MESENTRIALTROMBOSIS VENAEMBOLI PARU
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Dangers of Arterial Thrombosis
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PENATALAKSANAANPENGOBATAN
PRINSIP P.DARAH DIPERBAIKI MANIPULASI KOMPOSISI DARAH
BEDAH TROMBEKTOMI ARTERI BYPASS
MEDIS TROMBOLISIS ANTIKOAGULAN ANTIAGREGASI TROMBOSIT
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PENCEGAHANMENGHILANGKAN FAKTOR RISIKO
DIDAPAT HIPERTENSI ATEROSKLEROSIS DISPLIPIDEMIA OBAT KONTRASEPTIVE ORAL MEROKOK
MENGATASI AKIBAT DEF. AT III DEF. PROT C DEF. PROT S DISFIBRINOGENEMIA
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T E R I M A K A S I H