基因重組人類紅血球生成素所引起之純紅血球再生不良是一種罕見疾病，在此我們報告馬偕醫院確診的2 個基因重組人類紅血球生成素所引起之純紅血球再生不良的病例。病例一為一位五十三歲尿毒症並接受規則洗腎的女性，平時接受規律的紅血球生成素a(Eprex) 皮下注射。在Eprex 使用13 個月後病人被發現有不明原因且逐漸惡化的貧血。骨髓抽吸檢查顯示紅血球系列再生不良，且符合純紅血球再生不良的診斷。抗紅血球生成素抗體亦被偵測出。在停用Eprex 且使用環孢靈治療後，病人的貧血逐漸改善。且病人血液中亦偵測不到抗紅血球生成素抗體。之後給病人使用另一種類的紅血球生成素(darbepoetin) 治療。病人最後貧血完全復原。病例二是一位46 歲的男性，因慢性腎病引起的貧血接受規則的紅血球生成素b(Recormon) 皮下注射治療。儘管使用更高劑量的Recormon 以及darbepoetin 治療，他的貧血卻越來越惡化。病人血液中可偵測到抗紅血球生成素抗體。因此我們停止使用紅血球生成素以及使用免疫抑制的藥物類固醇和azathioprine 治療。病人貧血逐漸改善而不需要輸血。

Recombinant human erythropoietin (rHuEPO) induced antibody-mediated pure red cell aplasia (PRCA)was a rare disease. Herein, we reported 2 cases with confirmed diagnosis of EPO- induced antibodymediated PRCA in our institution. Case 1: a 53 year-old female with uremia and regular hemodialysis was regularly administered with EPO-α (Eprex)subcutaneously. Progressive unexplained anemia was noted 13 months after therapy with Eprex. Bone marrow study revealed remarkable erythroid hypoplasia compatible with PRCA. Anti-EPO antibody levels were detected. After withdrawal of Eprex and administration of cyclosporine,her anemia gradually improved. Serum level of anti-EPO antibody became undetectable. Another form of EPO (darbepoetin) was administered to the patient and hemoglobin recovered to 10.4 g/dL. Case 2:a 46 year-old male with chronic kidney disease related anemia underwent a regular subcutaneous EPO-β
(Recormon) therapy. He developed profound anemia in spite of dose increase of EPO-β and combination with darbepoetin usage. EPO induced antibody-mediated PRCA was confirmed by the detection of anti-EPO
antibody and severe erythroid hypoplasia in the bone marrow. Cessation of EPO and initiation of immunosuppression therapy with methylprednisolone and azathioprine were instituted. The patient finally became transfusion-independent.