造血干细胞移植后EB病毒相关性淋巴增殖性疾病一例分析

Successful treatment of post-transplant lymphoproliferative disorder in allogeneic hemopoietic stem cell transplant recipient for severe aplastic anemia

目的研究异基因造血干细胞移植后EB病毒(EBV)相关性淋巴增殖性疾病(EBV- PTLD)的早期诊断和治疗措施。方法1例16岁极重型再生障碍性贫血患者在用环磷酰胺、兔抗人胸腺细胞球蛋白和甲泼尼龙(CY/ATG/MP)预处理后行HLA1/6位点不合的同胞异基因造血干细胞移植。结果移植后72d发生EBV-PTLD。经及时停用环孢素A(CsA),同时加用丙种球蛋白、α-干扰素及MP治疗,患者病情好转。结论PTLD为造血干细胞移植和实体器官移植后一种少见但致命的并发症,高危患者应注意监测EBV-DNA变化、早期诊断、及时减少或停用免疫抑制剂尤为重要。

Objective To study early diagnosis and treatment of post-transplant lymphoproliferative disorder in allogeneic hemopoietic stem cell transplant recipients. Methods A 16 years old patient with severe aplastic anemia received HLA-mismatched sibling allogeneic hemopoietic stem cell transplant after conditioning with cyclophosphamide/ antithymocyte globulin/methylprednisolone （CY/ ATG/MP） regimen. Results On the day 72 posttransplantation, he developed lymphoproliferative disorder, After withdrawal of CsA, he was treated with methylprednisolone, intravenous immune globulin and IFN alpha, and recovered completely from PTLD. Conclusions PTLD is a rare and fatal complication of both solid-organ and hemopoietic stem cell transplantation. Surveillance for PTLD by PCR for circulating EBV-DNA may be appropriate in high risk settings. Early diagnosis, immunosuppression therapy reduction or even withdrawal in time is important.