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Azathioprine引起的單純紅血球再生不良

Azathioprine-induced Pure Red Cell Aplasia

摘要


Azathioprine為一免疫抑制劑,廣泛使用於器官移植或自體免疫疾病的病患。骨髓抑制是azathioprine眾所週知的副作用,其他如巨紅血球症(macrocytosis)及輕微的白血球減少症(leucopenia)也很常見,較少見的副作用包括貧血、血小板減少(thrombocytopenia)及全血球減少(pancytopenia)。單純紅血球再生不良(pure red cell aplasia; PRCA)則為azathioprine相當罕見的副作用。本文報告一位二十歲患有全身性紅斑性狼瘡的女性病息,使用azathioprine治療後,產生血紅素(Hb)降低的情形,經住院進行血液生化及骨髓組織檢查,懷疑是藥物所引起的單純紅血球再生不良。由於azathioprine產生的骨髓抑制作用大多使紅血球、白血球及血小板皆降低,單獨抑制紅血球的發生率極低,因此藉此病例報告討論其成因、可能性及因應處理方式。

並列摘要


Long-term administration of azathioprine is widely used to prevent rejection in organ transplantation and in the treatment of autoimmune diseases. Bone marrow toxicity is a well-known side-effect of azathioprine, which is frequently manifested as macrocytosis, with or without megaloblastic erythropoiesis in the bone marrow, and mild leucopenia. Less common effects are anemia, thrombocytopenia and pancytopenia. Pure red cell aplasia is a rare complication of azathioprine. A 20-year-old female was diagnosed as SLE with lupus nephritis. She has taken steroid, azathioprine, hydroxychloroquine and quinapril for SLE and received regular follow-up in our OPD. Because the Hb value was lowered to 6.9 mg/dL, this patient was admitted for studying the etiology of anemia. After admission, iron deficiency anemia was ruled out by normal ferritin level, and hemolytic anemia was also ruled out by negative finding on direct Coombs' test and normal LDH and bilirubin level. Bone marrow biopsy was conducted, which showed erythroid hypoplasia. We will review case reports in the literature and discuss the association and management of azathioprine-induced pure red cell aplasia.

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