HEMOSTASIS DAN KELAINAN HEMORRHAGIK

Rahajuningsih DharmaDepartemen Patologi Klinik

FKUI-RSCM

Definisi

• Hemostasis adalah mekanisme

tubuh untuk menghentikan

perdarahan dan mencegah

perdarahan spontan

Haemostasis

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

Fungsi trombosit

Yang berperan dalam hemostasis

1. Vaskular (vaso konstriksi)

2. Trombosit (sumbat trombosit)

3. Sistem koagulasi (fibrin)

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

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

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

+

Penyebab perdarahan

1. Kelainan vaskular

2. Kelainan trombosit

3. Kelainan sistem koagulasi

Pemeriksaan hemostasis

• Tujuan : untuk mencari penyebab

perdarahan

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

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

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)

Penyebab hemorrhagic diathesis

Vascular disorders

Platelet disorders

Coagulation disorders

Vascular disorders

Etiology :

Malformation of structure

Inflammatory process or immunologic reaction

Abnormality in perivascular tissues

Classification : Hereditary abnormalities

Acquired disorders

Hereditary abnormalities

Hereditary hemorrhagic telangiectasia

Ehlers-Danlos syndrome

Osteogenesis imperfecta

Pseudoxanthoma elasticum

Acquired vascular disorders

Henoch Schonlein syndromeSenile purpuraSteroid purpuraSimplex purpuraScurvyDrug-induced purpuraPurpura associated with infectionMechanical purpuraPurpura associated with paraproteinemia

Simplex purpura

Women during menstrual period

The cause is unclear

Tourniquet test and bleeding time : normal

Scurvy

Cause : vitamin C deficiency Collagen formation disturbed Increase vascular fragility Perifollicular petechiae Bleeding time : prolonged Tourniquet test : positive

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

Thrombocytosis

Physiologic thrombocytosis : epinephrine, exercise

Pathologic thrombocytosis :

primary thrombocytosis (thrombocythemia)

secondary thrombocytosis (reactive thrombocytosis)

Hereditary platelet function disorder

Glanzmann’s thrombasthenia

Bernard Soulier syndrome

von Willebrand’s disease

Disorder of release reaction

Hereditary Coagulation disorders

X-linked recessive

autosomal dominant

autosomal recessive

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 %

Laboratory diagnosis of Hemophilia A

Platelet count Bleeding time normal PT TT APTT : prolonged F VIII activity low von Willebrand’s factor normal

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 %

Laboratory diagnosis of Hemophilia B

Platelet count Bleeding time normal PT Thrombin Time APTT : prolonged F IX activity low

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

Acquired coagulation disorders

deficiency of vit. K dependent factors

liver disease

renal disease

pathologic anticoagulant

disseminated intravascular coagulation

fibrinogenolysis