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Question 9#

A 25-year-old woman complains of persistent bleeding for 5 days after a dental extraction. She has noticed easy bruisability since childhood, and was given a blood transfusion at age 17 because of prolonged bleeding after an apparently minor cut. She denies ecchymoses or bleeding into joints. Her father has noticed similar symptoms but has not sought medical care. Physical examination is normal except for mild oozing from the dental site. She does not have splenomegaly or enlarged lymph nodes. Her CBC is normal, with a platelet count of 230,000. Her prothrombin time is normal, but the partial thromboplastin time is mildly prolonged. The bleeding time is 12 minutes (normal 3-9 minutes).

What is most appropriate way to control her bleeding? 

a. Factor VIII concentrate
b. Fresh frozen plasma
c. Desmopressin (DDAVP)
d. Whole blood transfusion
e. Single donor platelets

Correct Answer is C

Comment:

This woman’s lifelong history of excessive bleeding suggests an inherited bleeding problem, as does the positive family history. The prolonged PTT indicates a deficiency of factors VIII, IX, XI, or XII, but the commonest of these deficiencies (classic hemophilia A and Christmas disease, or hemophilia B) are vanishingly rare in women. Furthermore, the continued oozing from dental sites and the absence of ecchymoses or hemarthroses suggest a platelet function disorder, as does the prolonged bleeding time. Von Willebrand disease is an autosomal dominant condition that leads to both platelet and factor VIII dysfunction and is the likeliest diagnosis in this patient. Although factor VIII concentrates can be used for life-threatening bleeding, most will respond to desmopressin, which raises the von Willebrand factor level in the most common form (the so-called type 1 form) of this disease. Mild von Willebrand disease is fairly common (1 in 250 individuals). Freshfrozen plasma and whole blood are much less effective ways to deliver factor VIII. Platelet transfusion would not be as effective as correction of the von Willebrand factor level.