USMLE (Fach) / Hematology and Oncology (Lektion)
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Hemophilia A, B, or C
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Intrinsic pathway coagulation defect (↑ PTT).A: deficiency of factor VIII → X-linked recessiveB: deficiency of factor IX → X-linked recessiveC: deficiency of factor XI → autosomal recessive- Normal bleeding time (no inherent platelet disorder)
Hemorrhage in hemophilia – hemarthroses (bleeding into joints, eg, knee), easy bruising, bleeding after trauma or surgery (eg, dental procedures).
Treatment: desmopressin + factor VIII concentrate (A), factor IX concentrate (B), factor XI concentrate (C).
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