HEMOSTASIS DAN KELAINAN HEMORRHAGIK

Rahajuningsih Dharma Departemen Patologi Klinik FKUI-RSCM

Definisi
Hemostasis adalah mekanisme tubuh untuk menghentikan perdarahan dan mencegah perdarahan spontan

Haemostasis

Vascular injury

tissue thromboplastin

platelet adherent ADP

vaso constriction

F XII activation

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. 2. 3. Vaskular (vaso konstriksi) Trombosit (sumbat trombosit) Sistem koagulasi (fibrin)

Sistem koagulasi
Factor I Factor II Factor III Factor IV Factor V Factor VII Factor VIII Fibrinogen Prothrombin Thromboplastin jaringan Calsium ion Proaccelerin Proconvertin Anti hemophilic factor

Sistem koagulasi
Factor IX Christmas factor

Factor X
Factor XI

Stuart Prower factor

Antihemophilic C

Factor XII
Factor XIII

Hageman factor
Fibrin stabilizing factor

Prekallikrein

Fletcher factor

HMW kininogen Fitzgerald factor

Negative surface Kal. PK HMWK XII XIIa XI IX XIa IXa Ca++ VIII Pf 3 VIIIa X Pf3 Va Prothrombin Xa Ca++

Tissue thromboplastin

VIIa

VII

Ca++

F1.2

Fibrinogen

+ Thrombin
Fibrin monomer Fibrin polimer XIIIa

FPA FPB

XIII

Stabilized fibrin

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: Khusus: Masa trombin Hitung trombosit Fibrinogen Uji pembendungan Agregasi trombosit M. Perdarahan D dimer Masa protrombin PT F VIII, F IX Antitrombin III Masa tromboplastin parsial teraktivasi APTT Protein C dan S Thrombotest Masa pembekuan Lupus 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 syndrome Senile purpura Steroid purpura Simplex purpura Scurvy Drug-induced purpura Purpura associated with infection Mechanical purpura Purpura 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

Glanzmanns thrombasthenia Bernard Soulier syndrome von Willebrands 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 Willebrands 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 Willebrands 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