紅血球生成素引起之抗體導致的純紅血球再生不良：一病例報告

Epoetin-Related Antibody-Mediated Pure Red Cell Aplasia (PRCA): A Case Report

黃千惠(Chien-Huei Huang) ； 王明誠(Ming-Cheng Wang) ； 高雅慧(Yea-Huei Kao Yang) ； 莊善安(Shan-An Chuang) ； 黃群超(Chun-Chao Huang) ； 張孔昭(Kung-Chao Chang) ； 黃建鐘(Jeng-Jong Huang)

《內科學誌》 20卷1期 (2009/02) Pp. 86-91

https://doi.org/10.6314/JIMT.2009.20(1).12

摘要

基因重組人類紅血球生成素(Recombinant human erythropoietin, rHuEpo, epoetin)所造成之純紅血球再生不良(pure red cell aplasia, PRCA)是發生於腎性貧血患者的一種罕見血液疾病，其臨床特徵是使用rHuEpo治療時，仍有嚴重貧血。血液檢查方面，除紅血球母細胞數目偏低外，其餘的血球和數目均爲正常，推測可能致病機轉與製劑之賦型劑成份和注射投予的方式有關。目前的全球發生的案例數大多爲使用epoetin-α劑型，其他的劑型（如：epoetin-β）較少。目前文獻探討改變賦型劑成份或注射投予的方式，可降低PRCA病例。本病例爲一接受血液透析的腎性貧血女性透析患者，使用epoetin-β製劑後，發生了嚴重貧血，經由骨髓抽吸檢查與血清中epoetin的中和性抗體檢驗，顯示爲由epoetin造成之純紅血球再生不良。該患者併有糖尿病，在停止使用epoetin和接受類固醇治療一星期後，因血糖難以控制和對類固醇的耐受性差而停止使用，之後改用mycophenolate mofetil (MMF)治療三個月，但未見良好療效，後不幸死於敗血性休克與肝衰竭。所以，腎性貧血患者臨床使用epoetin時，若施打epoetin-α，應以靜脈方式投予；若使用期間，患者的貧血未改善並出現加重的情形，則需作進一步相關性檢查（如：骨髓抽吸和中和性抗體）以確定是否爲epoetin造成的純紅血球再生不良，再行適當之治療。

關鍵字

腎性貧血 ； 紅血球生成素 ； 基因重組人類紅血球生成素 ； 純紅血球再生不良 ； 賦型劑 ； 穩定劑

並列摘要

Epoetin-related antibody-mediated pure red cell aplasia (PRCA) is a rare hematological disorder in patients with renal anemia. This disorder results in progressively severe anemia even under epoetin therapy. The conformational investigations of PRCA include normal cellularity and the number of erythroblasts was lower than 5%. The immunological mechanism of developing antibody-mediated PRCA is unclear, but a certain of factors may increase the risk of immunogenicity, including the components and properties of product formulation and route of administration. The majority of cases of epoetin-induced PRCA were associated with use of formulation of epoetin-α, and the other formulation associated with the PRCA was relatively lower. This 48-year-old uremic female received hemodialysis therapy and subcutaneous (sc) injections of epoetin-β for her renal anemia. But she still showed severe anemia (hemoglobin around 7g/dl) in spite of frequent blood transfusion 2 wks. After bone marrow aspiration and serum neutralizing IgG antibody examinations, the diagnosis of PRCA was confirmed. Subsequently, we discontinue the use of Epo and treat her with corticosteroid. Because she had diabetic history and can't tolerate the adverse effects of steroid. Since March 2006, she received mycophenolate mofetil (MMF) therapy for three months. But her anemia did not improve, and other immunotherapy is necessary. Unfortunately, she died of septic shock and liver failure. In brief, we need further examinations to confirm the underlying causes of PRCA and receive appropriate immunotherapy or kidney transplantation if dialysis patients on Epo therapy became transfusion-dependent

並列關鍵字

Renal anemia ； Erythropoietin ； Recombinant human erythropoietin, rHuEpo, epoetin ； Pure red cell aplasia, PRCA ； Excipient ； Stabilizer