면역억제 치료 후 Darbapoetin에 반응한 진성 적혈구 무형성증
Successful Rechallenge with Darbepoetin Following Immunosuppressive Therapy in a Dialysis Patient with Erythropoietin-Induced Pure Red Cell Aplasia
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초록
항 EPO 항체에 의한 PRCA는 매우 드물지만 적절한 치료를 하지 않으면 지속적인 적혈구 수혈이 필요하게 되므로문제가 된다. PRCA가 진단되면 ESA 투여를 중단하고 면역억제 치료를 하는 것이 필요하다. 특히 항 EPO 항체는 모든종류의 ESA 제제와 교차반응을 하므로, 어떤 ESA 제제로도교체 투여하지 않아야 한다. 국내에서는 3예의 증례가 보고되었는데, 면역억제 치료를 시행하였으나 모든 예에서 치료에 성공하지 못하였다. 저자들은 EPO-α를 피하 주사하던 환자에서 발생한 PRCA를 면역억제 치료 후에 darbepoetin-α로교체 투여하여 성공적으로 치료한 예를 경험하였기에 문헌고찰과 함께 보고한다.
Abstract
Patients with erythropoiesis-stimulating agent (ESA)-induced pure red cell aplasia (PRCA) should not routinely be switched to an alternative ESA or to darbepoetin-α because anti-erythropoietin (anti-EPO) antibodies cross-react with all kinds of recombinant ESAs. We present a case of ESA-induced PRCA in a 69-year-old man on hemodialysis whose anemia improved with reintroduction of darbepoetin-α following immunosuppressive therapy. The patient developed severe anemia after 15 months of subcutaneous administration of erythropoietin-α. After the diagnosis of PRCA, erythropoietin-α was discontinued and immunosuppressive therapy with a combination of prednisolone and oral cyclophosphamide was initiated. After 4 months of immunosuppressive therapy, the anti-EPO antibody titer was markedly decreased; however, esophageal candidiasis developed. Additional therapy with cyclosporine alone instead of prednisone and cyclophosphamide was performed, and anti-EPO antibody was subsequently not detected. Darbepoetin-α was then reintroduced, and the patient’s anemia improved without red cell transfusion. In conclusion, ESA-induced PRCA was successfully treated with reintroduction of darbepoetin-α following immunosuppressive therapy.
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