Abstract

Thrombotic thrombocytopenic purpura (TTP) is a thromboembolic disorder that has been associated with oral contraceptive (OC) use. This discussion describes a patient who developed TTP subsequent to treatment with high dose OCs during treatment of hirsutism. The patient a 22-year old nulligravid woman presented for evaluation of hirsutism which began at puberty in association with mild acne and irregular menses. An endocrine evaluation demonstrated elevated testosterone 0.91 ng/ml; elevated free testosterone 2.65%; and elevated dehydroipiandrosterone 11.4 ng/ml. Partial suppression of the androgens with both dexamethasone and OCs was demonstrated. Prednisone 5 mg daily and ethinyl estradiol 50 mg and ethynodiol acetate (Demulen) were prescribed. This treatment was continued uneventfully for 1 year with improvement in the patients symptoms. 5 weeks prior to hospital admission she was prescribed mestranol with norethindrone (Ortho-Novum 1/80) because of hypomenorrhea and occasional amenorrhea. 3 days prior to admission the patient noted petechiae on her trunk and extremities. Except for moderate hirsutism nonpalpable purpura and ecchymoses of wide areas of the skin the physical examination on admission was unremarkable. Laboratory data demonstrated thrombocytopenia and intravascular hemolytic anemia. Early in the patients clinical course she developed confusion slurred speech and right hemiparesis all of which resolved within 48 hours. The bone marrow contained an increased number of megakarocytes with moderately increased erythroid cellularity. Biopsies of skin muscle and bone marrow failed to demonstrate evidence of intravascular thrombi suggestive of thrombocytopenic purpura. Corticosteroids and plasma infusions were initiated but failed to control the disease. The patient ultimately responded to plasma exchanges in combination with aspirin dipyridamole and prednisone with control of the consumptive thrombocytopenia and microangiopathic anemia. She suffered relapse when the exchanges were discontinued after 4 weeks of therapy and required 2 additional weeks of plasma exchanges to establish sustained remission. The patient is currently receiving prednisone 5 mg daily for hirsutism. The clinical presentation of TTP is variable but includes the microangiopathic hemolytic anemia thrombocytopenia and fluctuating neurologic signs and renal failure. Failure of other organs may also occur. The etiology of the disease remains obscure. The most efficacious treatment appears to be plasma infusion or exchanges.

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