A47-year-old white male was referred for chronic thrombocytopenia of 80-90 X 109/L and splenomegaly since 1997. He had been treated unsuccessfully, and mistakenly, for immune thrombocytopenia (steroids, rituximab, romiplostim, and anti-D that produced severe hemolysis requiring hospitalization). Previous bone marrow biopsies were nondiagnostic and an earlier liver biopsy showed steatosis. A cholecystectomy had been performed for gallstones. Family history was negative. In addition to mild splenomegaly, he presented with xanthomas. Labs showed hemoglobin 12.3 g/dL, haptoglobin 2 mg/dL, indirect bilirubin 2.4 mg/dL, and an elevated absolute reticulocyte count 240 mil/L. Direct antiglobulin test and flow cytometry for paroxysmal nocturnal hemoglobinuria were negative.
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机译：自1997年以来，一名47岁的白人男性因慢性血小板减少症80-90 X 109 / L和脾肿大而被转诊。他因免疫性血小板减少症（类固醇，利妥昔单抗，romiplostim和产生的抗D）治疗失败，但误诊为严重溶血需要住院）。先前的骨髓活检未确诊，较早的肝活检显示脂肪变性。胆囊结石已进行了胆囊切除术。家族史为阴性。除轻度脾肿大外，他还出现了黄瘤。实验室显示血红蛋白为12.3 g / dL，触珠蛋白为2 mg / dL，间接胆红素为2.4 mg / dL，绝对网织红细胞计数为240 mil / L。阵发性夜间血红蛋白尿的直接抗球蛋白测试和流式细胞仪阴性。
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