PIS spontan terkait gangguan hematologi

PIS spontan terkait gangguan hematologi2 kategori besarDidapat (Acquired)Dari lahir (congenital)Didapat (Acquired)Drug (antiplatelet, anticoagulant, thrombolytic)Thrombocytopenia

Coagulation cascade, the traditional concept

FibrinolysisComponents of the fibrinolytic system:- plasminogen and plasmin The fibrinolytic system

Plasminogen activators:

- tissue plaminogen activator (tPA)- urinary plaminogen activator (uPA)- exogenous plasminogen activators (streptokinase)

2ITP, dan penyakit lainnya termasuk purpura trombotik trombositopenik, posttransfusion purpura, dan DIC; 3) penyerapan abnormal, biasanya dalam limpa, seperti pada sirosis; dan 4) beberapa penyebab, seperti biasa terlihat pada pecandu alkohol. Kasus trombositopenia yang diinduksi PIS telah dikaitkan dengan penggunaan obat-obatan tertentu, serta uremia, penggunaan alkohol, dan transplantasi hati. 9Congenital Coagulation Disorders

Heparin is a cofactor that allows antithrombin III to inactivate thrombin and Factor XaThrombomodulin binds to thrombin, making it an anticoagulant whichthen activates anti-coagulant protein C.Protein C cleave factors Va and VIIIaHemophilia B (ChristmasDisease) results from deficiency of factor IXHemophilia A (classic)is due to reduced amount or reduced activity of Factor VIII Coagulation DisordersLaboratory findings:Normal bleeding time & Platelet countProlonged prothrombin time (PT)deficiencies of II, V, VII, XProlonged time (aPTT)all factors except VII, XIIIMixing studies - normal plasma corrects PT or aPTT

Factor VIII DeficiencyClassic hemophilia (hemophilia A):

X-linked disorder (affects 1 males)Most common - severe bleedingSpontaneous hematomas < 1, 5, 75%Abnormal aPTT Intrinsic path.Diagnosis - factor VIII assayTreatment - factor VIII concentrateCryoprecipitate (less desirable)Concentrat factor VIII KoateRecombinat factor VIII

Factor IX DeficiencyChristmas disease (Hemophilia B):

X-linked recessive disorder Indistinguishable from classic hemophilia (F VIII)Requires evaluation of factor VIII and IX activity levels to diagnoseTreatment - factor IX concentrateCryoprecipitate if factor IX unavailable

Von-Willebrand Disease:Coagulation + PLT disorder:

Congenital disorderDeficiency of vWF molecule Part of FVIII, Mediates platelet adhesionProlonged Bleeding timeLow Factor VIII & long aPTTMucocutaneous bleeding

Von-Willebrand Disease:

vWF: F-VIII & Plt function.Defective Platelet AdhesionSkin Bleeding Prolonged Bleeding time.Low Factor VIII levels.12/05/2015

Terima Kasih..