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A 2 2 -year-old male patien t presents with recu rrent epistaxis and a history of pro-longed bleeding after m i nor cuts. At age LJ, he had a splenectomy due to presumed immune th rombocytopenia. He is referred to hematology for evaluation of thrombo-cytopenia. His platelet count is LJ7 .000/ JLL. Hemoglobin and leukocyte coun ts are normal. A peripheral blood smear is shown below.
What treatment has limited effectiveness in the management of Bernard-Soulier syndrome?
- Platelet transfusion
- Antifibrinolytic agents
- DDAVP (desmopressin)
- Bone marrow transplantation
Medicine
C. DDAVP is more useful in the treatment of platelet function defects than it is in conditions involving platelet receptor deficiency. This does not preclude a trial of desmopressin in BSS patients. Plate-let transfusions are an integral part of the acute management of bleeding episodes but alloimmunization is a serious complication. Antifibrinolytics are useful for minor bleeding and prevention of postprocedure bleeding. Bone marrow transplantation has been successful in several patients with Bernard-Soulier syndrome. Recombinant factor VII has also been ofbenefit (Br l Haematol. 2010; 149:81 3-823).
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