Features of Von Willebrand disease are –
Correct Answer: | BT and | PTT
Description: Ans. is 'a' i.e., | BT and | PTT Von willebrand diseaseo Von willebrand disease is characterized by the deficiency of vWF, while the synthesis of factor VIIIc is normal,o vWF has two major function: -i) Helps in platelet adhesionii) Stabilizes factor VIIIc.o So, deficiency of vWF results in manifestation of both.Platelet adhesion defecto Bleeding from small venules and capillaries of skin and mucous membrane e.g., epistaxis.o Menorrhagia and GI bleeding,o Proioged bleeding time (TBT)Coagulation defect (due to factor VIII dysfunction)o Bleeding into weight bearing joints.o Large post traumatic ecchymoses or hematoma,o Prolonged bleeding after any form of surgical procedure or a laceration,o Prolonged PTT.o vWF can be assayed by restocetin agglutination test which measures the ability of restocetin to promote the interaction between vWF and platelet membrane glycoprotein Ib-IX.o Von willebrand disease is of follow ing types: -Type I (most common 70%) - Autosomal dominant, with reduced quantity of vWF.Type II (25%) - Autosomal dominant, with qualitative defect of vWF.Type III - Autosomal recessive, with reduced quantify of vWF.Hemophilia A & Hemophilia B|PTT Normal BT Normal PTVon-Willebrand disease| PTT |BT Normal PTPlatelet dysfunction| BTo Bemard-soulier syndrome o Glanzmann's thrombastheniaNormal PT & PTTo ITP Henoch Schonlein purpuraNormal BT, PT & PTT
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