Hemostasis Dan Kelainan Hemorrhagik Utk Fkg
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Transcript of Hemostasis Dan Kelainan Hemorrhagik Utk Fkg
HEMOSTASIS DAN KELAINAN HEMORRHAGIK
Rahajuningsih DharmaDepartemen Patologi Klinik
FKUI-RSCM
Definisi
• Hemostasis adalah mekanisme
tubuh untuk menghentikan
perdarahan dan mencegah
perdarahan spontan
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