hemostasis dan kelainan hemorrhagik utk fkg
TRANSCRIPT
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HEMOSTASIS DAN KELAINAN HEMORRHAGIK
Rahajuningsih DharmaDepartemen Patologi Klinik
FKUI-RSCM
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Definisi
• Hemostasis adalah mekanisme
tubuh untuk menghentikan
perdarahan dan mencegah
perdarahan spontan
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Vascular injury
tissue thromboplastin platelet adherent vaso constriction
F XII activation ADP
primary platelet aggregation
PF3 ADP 5 OH tryptamin TxA2
secondary platelet aggregation (semi permeable platelet plug) fibrin
non permeable platelet plug
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Fungsi trombosit
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Yang berperan dalam hemostasis
1. Vaskular (vaso konstriksi)
2. Trombosit (sumbat trombosit)
3. Sistem koagulasi (fibrin)
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Sistem koagulasi Factor I Fibrinogen
Factor II Prothrombin
Factor III Thromboplastin jaringan
Factor IV Calsium ion
Factor V Proaccelerin
Factor VII Proconvertin
Factor VIII Anti hemophilic factor
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Sistem koagulasi Factor IX Christmas factor
Factor X Stuart Prower factor
Factor XI Antihemophilic C
Factor XII Hageman factor
Factor XIII Fibrin stabilizing factor
Prekallikrein Fletcher factor
HMW kininogen Fitzgerald factor
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Negative surface Tissue thromboplastin Kal. PK HMWKXII XIIa
XI XIa VIIa VII
IX IXa Ca++
Pf 3 VIII VIIIa
X Xa Ca++
Pf3
V Va Fibrinogen
Prothrombin ThrombinFibrin monomer
Fibrin polimer
XIII XIIIa
Stabilized fibrin
Ca++
FPA
FPB+
F1.2
+
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Penyebab perdarahan
1. Kelainan vaskular
2. Kelainan trombosit
3. Kelainan sistem koagulasi
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Pemeriksaan hemostasis
• Tujuan : untuk mencari penyebab
perdarahan
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Indikasi pemeriksaan hemostasis
• Pasien dengan gejala perdarahan: petekiae, ekimosis, hematoma, hemartrosis, hematuria, menorrhagia
• Riwayat perdarahan• Pre operasi• Dugaan hemostasis terganggu :sirosis
hati, sepsis• Pemantauan terapi heparin,
antikoagulan oral
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Pemeriksaan hemostasis
Penyaring:
Hitung trombosit
Uji pembendungan
M. Perdarahan
Masa protrombin PT
Masa tromboplastin parsial teraktivasi APTT
Masa pembekuan
Khusus:Masa trombinFibrinogenAgregasi trombositD dimerF VIII, F IXAntitrombin IIIProtein C dan SThrombotestLupus anticoagulant
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Persiapan pasien
• Puasa : tidak mutlak
• Makanan : sayuran hijau (vit K INR), ginseng, bawang, jamur, rumput laut (agregasi trombosit)
• Obat-obatan: aspirin, NSAID (agregasi trombosit) obat yang mempengaruhi warfarin INR
• Exercise: sistem fibrinolisis (t-PA)
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Penyebab hemorrhagic diathesis
Vascular disorders
Platelet disorders
Coagulation disorders
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Vascular disorders
Etiology :
Malformation of structure
Inflammatory process or immunologic reaction
Abnormality in perivascular tissues
Classification : Hereditary abnormalities
Acquired disorders
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Hereditary abnormalities
Hereditary hemorrhagic telangiectasia
Ehlers-Danlos syndrome
Osteogenesis imperfecta
Pseudoxanthoma elasticum
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Acquired vascular disorders
Henoch Schonlein syndromeSenile purpuraSteroid purpuraSimplex purpuraScurvyDrug-induced purpuraPurpura associated with infectionMechanical purpuraPurpura associated with paraproteinemia
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Simplex purpura
Women during menstrual period
The cause is unclear
Tourniquet test and bleeding time : normal
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Scurvy
Cause : vitamin C deficiency Collagen formation disturbed Increase vascular fragility Perifollicular petechiae Bleeding time : prolonged Tourniquet test : positive
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The classification of thrombocytopenia
Decrease production:Aplastic anemia
Leukemia
Megaloblastic anemia
Increase destruction:Idiopathic thrombocytopenic purpura
Drug-induced thrombocytopenia
Increase consumption: Disseminated intravascular coagulation
Thrombotic thrombocytopenic purpura
Increase pooling: splenomegali Dilution: massive transfusion with stored blood
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Thrombocytosis
Physiologic thrombocytosis : epinephrine, exercise
Pathologic thrombocytosis :
primary thrombocytosis (thrombocythemia)
secondary thrombocytosis (reactive thrombocytosis)
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Hereditary platelet function disorder
Glanzmann’s thrombasthenia
Bernard Soulier syndrome
von Willebrand’s disease
Disorder of release reaction
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Hereditary Coagulation disorders
X-linked recessive
autosomal dominant
autosomal recessive
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Hemophilia A
x-linked recessive affects man, women acts as carrier deficiency or dysfunction of F VIII hemarthrosis, hematoma, delayed bleeding severe : VIII < 1% moderate : VIII 1 - 5% mild : 5 - 20 %
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Laboratory diagnosis of Hemophilia A
Platelet count Bleeding time normal PT TT APTT : prolonged F VIII activity low von Willebrand’s factor normal
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Hemophilia B (christmas disease)
x -linked recessive Deficiency or dysfunction of F IX Clinical symptoms = hemophilia A severe : F IX < 1% moderate : F IX 1 - 5% mild : F IX 5 - 20 %
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Laboratory diagnosis of Hemophilia B
Platelet count Bleeding time normal PT Thrombin Time APTT : prolonged F IX activity low
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von Willebrand’s disease
autosomal dominant mucosal bleeding Deficiency or dysfunction of vWF bleeding time prolonged aggregation by ristocetin abnormal PT, TT normal APTT : normal or prolonged F VIII activity: normal or low
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Acquired coagulation disorders
deficiency of vit. K dependent factors
liver disease
renal disease
pathologic anticoagulant
disseminated intravascular coagulation
fibrinogenolysis