异基因造血干细胞移植后小脑淋巴细胞增殖性疾病一例报告并文献复习

Cerebella post transplant lymphoproliferative disease after allogeneic hematopoietic stem cell transplantation:one case report and literature review

目的探讨异基因造血干细胞移植术(hematopoietic stem cell transplantation, HSCT)后中枢神经系统EB病毒(EBV)相关淋巴细胞增殖性疾病(post transplant lymphoproliferative disease, PTLD)的诊断及治疗进展。方法收集航天中心医院1例HSCT后小脑PTLD病例的临床资料,分析相关临床及实验室检查特点并复习相关文献。结果 1例急性髓系白血病患者行HLA 5/10相合异基因HSCT,移植后+147 d出现意识丧失及抽搐,头颅MRI提示右侧小脑半球异常信号灶,行立体定向脑穿刺活检,病理结果为EBV相关的弥漫大B细胞淋巴瘤,行局部放射治疗。结论 PTLD是HSCT后一种少见但具有潜在致命性的合并症,原发于中枢神经系统少见,穿刺活检对于诊断具有重要意义。应根据患者具体情况给予个体化治疗,对于CD20^+的病例可考虑使用利妥昔单抗治疗。

Objective To investigate the diagnosis and progress of treatment of central nervous system(CNS) EpsteinBarr virus(EBV) associated post transplant lymphoproliferative disease(PTLD) after allogeneic hematopoietics stem cell transplantation(HSCT). Methods One case of cerebella PTLD after HSCT was reported. The relative clinical data and laboratory data were analyzed and the relevant literatures were reviewed. Results A patient with acute myeloid leukemia(AML)underwent HLA 5/10 allogeneic HSCT, and appeared loss of consciousness and convulsions on 147 d after transplantation.Head MRI indicated that there was an abnormal signal lesion in the right side cerebellar hemisphere. The patient received stereotactic brain puncture biopsy, pathology report showed EBV associated diffuse large B-cell lymphoma, and local radiotherapy was performed. Conclusions PTLD is a rare but potentially fatal complication of HSCT, which originated from CNS.Puncture biopsy is of great significance for diagnosis. Individualized treatment should be given according to the patient’s specific situation, and rituximab treatment should be considered for CD20 positive cases.