Name
HEMOPHILIA-B DISEASE
DESCRIPTION
DETAIL
DIFFERENTIAL DIAGNOSIS β’ von Willebrand disease β’ Vitamin K deficiency (factor IX is vitamin K dependent) β’ Other factor deficiencies, afibrinogenemia, dysfibrinogenemia, fibrinolytic defects, platelet disorders -------------------------------------------------------------------------- CAUSE : Inherited bleeding disorders due to deficiency of coagulant factor IX (hemophilia B) OTHER TESTS : * ASSAY OF FACTOR IX - DEFICIENT * BLEEDING TIME & PTT - PROLONGED β’ Activated partial thromboplastin time (PTT) is prolonged while platelet count, and prothrombin time are normal β’ PTT is corrected when mixed with normal plasm
TYPENOTES
RISK FACTORS Positive family history. Can predict risk to offspring using simple Mendelian genetics for an X-linked recessive disorder.PRECAUTIONS: Hemophilia B - some factor IX concentrates contain trace amounts of activated vitamin K-dependent factors and therefore are thrombogenic and carry a risk for thromboembolism
RELATED DISEASE
Not Available Disease
DISEASE
INVESTIGATION
PLATELET COUNT, BLEEDING TIME, CLOTTING TIME, PROTHROMBIN TIME, PARTIAL THROMBOPLASTIN TIME, COMPLETE BLOOD COUNT