成年人慢性活动性EB病毒感染相关T细胞淋巴瘤合并噬血细胞综合征一例并文献复习

Adult chronic active Epstein-Barr virus infection-related T-cell lymphoma complicated with hemophagocytic syndrome:report of one case and review of literature

目的探讨慢性活动性EB病毒感染(CAEBV)患者的病理变化机制。方法回顾性分析苏州弘慈血液病医院确诊的1例CAEBV相关T细胞淋巴瘤合并噬血细胞综合征患者的临床表现、实验室检查及诊治经过,并进行相关文献复习。结果结合不同时期实验室、淋巴结活组织检查及骨髓等检测结果,患者初期表现为淋巴组织增殖性疾病,逐步转变成淋巴瘤,具有不同时期EB病毒相关淋巴细胞克隆表现的诊断结果。患者在不同时期接受相应方案治疗,但最终因合并噬血细胞综合征且病情进展迅速死亡。结论CAEBV可能导致淋巴细胞由多克隆状态向单克隆逐步演变。

Objective To investigate the pathological change mechanism of patients with chronic active Epstein-Barr virus infection(CAEBV).Methods The clinical manifestations,laboratory examinations,diagnosis and treatment of one CAEBV patient in Suzhou Hongci Hematology Hospital of Jiangsu Province were retrospectively analyzed with review of related literature.Results Combined with the results of laboratory,lymph node biopsy and bone marrow tests in different periods,the patient initially showed lymphoproliferative disease,and gradually transformed into lymphoma with the diagnosis results of EB virus-related lymphocyte clones in different periods.The patient received the corresponding treatment plan in different periods,but eventually died due to hemophagocytic syndrome and rapid progress of the disease.Conclusion CAEBV may cause lymphocytes to gradually evolve from polyclonal to monoclonal state.