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Ann Hematol. 2004 Apr;83(4):253-5. Epub 2003 Sep 25.

Thrombotic thrombocytopenic purpura: a rare cause of thrombocytopenia in HIV-infected hemophiliacs.

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  • 1Department of Medicine, Long Island Jewish Medical Center, New Hyde Park, NY 11040, USA.


Thrombocytopenia is a common complication in human immunodeficiency virus (HIV)-infected hemophiliacs. The etiology is multifactorial and a majority of the patients with hemophilia exhibit a decreased platelet count within 10 years of seroconversion. Thrombocytopenia in these patients is associated with a high risk of bleeding and death. Thrombotic microangiopathy causing thrombocytopenia in HIV-infected hemophiliacs is extremely rare. We describe an HIV-infected hemophilic patient who presented with bleeding, renal insufficiency, and thrombocytopenia. Platelet transfusion resulted in deterioration of clinical condition. Examination of blood smears demonstrated a microangiopathic process. The patient responded well to plasmapheresis with normalization of platelet and renal function. Thrombotic thrombocytopenic purpura should be suspected in HIV-infected hemophiliacs who present with a new onset of thrombocytopenia and anemia as delay in treatment may result in fatal sequelae.

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