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

叶丽萍; 刘丽辉; 施兵; 胡文清; 张玉珠; 李国建; 刘明娟; 陈虎

doi:10.13201/j.issn.1004-2806.2012.02.011

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

- 解放军第309医院血液科(北京, 100091)

详细信息

通讯作者: 叶丽萍,E-mail:taitaiyang@126.com

中图分类号: R557

收稿日期: 2011-06-08

修回日期: 2011-08-04

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

- Department of Hematology, Chinese PLA Hospital No. 309, Beijing, 100091, China

More Information

Corresponding author: YE Liping, E-mail: taitaiyang@126.com

Received Date: 08 June 2011

Revised Date: 04 August 2011

摘要

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。
- 噬血细胞综合征,EB病毒相关 /
- 成人 /
- 免疫化学治疗
Abstract: Objective:To strengthen the understanding of adult EBV-associated hemophagocytic lymphohistiocytosis(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,pancytopenia hepatosplenomegaly and lymphadenhypertrophy as initial signs and symptoms and suffered ascites later.The course of the disease lasted sixteen months,pathological morphology and immunohistochemical studies revealed 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.
- EBV-associated hemophagocytic lymphohistiocytosis /
- adult /
- immunochemotherapy

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参考文献(16)

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