A young man presents with a history of easy bruising and prolonged bleeding after minor injuries. His maternal uncle also had a similar bleeding disorder. Laboratory investigations reveal a prolonged aPTT, with a normal PT. A mixing study corrects the aPTT. Which of the following is the most likely diagnosis?
سؤال مجاني 2 من 10 اليوم · اشترك وكمّل بدون حد
اعرض الإجابة
الإجابة والشرح
ادخل لحسابك عشان نحفظ إجاباتك ونوريك أخطاءك.
الإجابة: B. Hemophilia A
Easy bruising and prolonged bleeding in a young man whose maternal uncle is affected suggests X-linked inheritance. A prolonged aPTT with normal PT means an intrinsic-pathway factor problem, and correction on mixing means factor deficiency rather than an inhibitor. Together these fit haemophilia A (factor VIII deficiency).
- vWD is autosomal and typically presents with mucocutaneous bleeding.
- Factor VII deficiency prolongs the PT, not the aPTT.
- Antiphospholipid antibodies (lupus anticoagulant) do not correct on mixing.
ليش كل خيار صح أو غلط
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A
ليش غلط
vWD is autosomal (dominant or recessive), so it affects males and females alike, unlike haemophilia. A maternal-uncle pattern suggests X-linked inheritance.
✓ Hematology🩸.pdf · p. 38 -
B
ليش صح
Haemophilia: intrinsic-pathway factor problem (VIII, IX, XI) with a prolonged aPTT and normal PT, and the aPTT corrects on mixing because a factor is missing. Maternal-uncle history fits X-linked haemophilia A.
✓ Hematology🩸.pdf · p. 35 -
C
ليش غلط
Factor VII belongs to the extrinsic pathway, so its deficiency raises the PT/INR. Here the PT is normal and the aPTT is prolonged.
✓ HEME-06 Coagulopathy [Notes].pdf · p. 1 -
D
ليش غلط
With a clotting inhibitor such as lupus anticoagulant the aPTT stays prolonged and fails to normalize on mixing. Here the mixing study corrects, so a factor is deficient.
✓ Hematology🩸.pdf · p. 35
- المصدر
- Hematology🩸.pdf · p. 35
- المحاضرة
- Inherited Bleeding Disorders · Hematology · Module C
- ظهر في
- MED42 Compre C