成人EB病毒相关噬血淋巴组织细胞增生症1例并文献复习  

An adult case with EBV-associated hemophagocytic lymphohistiocytosis, report and review of the literature

作　　者：叶丽萍[1] 刘丽辉[1] 施兵[1] 胡文清[1] 张玉珠[1] 李国建[1] 刘明娟[1] 陈虎[1] 

机构地区：[1]解放军第309医院血液科,北京100091

出　　处：《临床血液学杂志》2012年第2期172-174,共3页Journal of Clinical Hematology

摘　　要：目的:提高对成人EB病毒相关的噬血细胞综合征(EBV-HLH)的认识,探讨适合的治疗方法。方法:报告1例长期发热的成人EBV-HLH病例的临床表现、检查与病理和治疗转归结果,结合文献进行分析讨论。结果:本例成人EBV-HLH患者以反复发热伴全血细胞减少,肝脾、淋巴结肿大为首发症状,继而出现腹水,病程16个月,病理形态学及免疫组织化学提示淋巴细胞增殖性疾病———T细胞淋巴瘤待查,EBV(+)。用VP-16联合激素及EBV-CTL治疗获得缓解。结论:成人EBV-HLH少见,应重视与淋巴瘤的鉴别诊断,积极合理地治疗可挽救患者的生命。Objective：To strengthen the understanding of adult EBV-associated hemophagocytic lymphohistio cytosis（EBV HLH）and explore appropriate therapeutic method. Method：With clinical manifestations, laboratory data,pathological findings and therapeutic outcome and prognosis reported, an adult case diagnosed with EBV- HLH was investigated in addition to the review of literature. Result：The adult patient had a recurrent fever, pancy- topenia hepatosplenomegaly and lymphadenhypertrophy as initial signs and symptoms and suffered ascites lat- er. The course of the disease lasted sixteen months,pathological morphology and immunohistochemical studies re- vealed lymphocytic proliferative disease --T cell lymphoma with positive EBV. Treatment with Vp 16 combined with a corticosteroid ＆ autologous EBV-specific Cytotoxic T lymphocytes induced a remission. Conclusion：The occurrence of EBV-HLH is scarce in adults. Special attention should be paid to the differential diagnosis between the disease and malignant lymphoma. Correct and prompt treatment is essential to save the patient.

关 键 词：噬血细胞综合征 EB病毒相关 成人 免疫化学治疗 

分 类 号：R557[医药卫生—血液循环系统疾病]