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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 improve the understanding of EBV-related hemophagocytic syndrome (EBV-HLH) in adults and to explore suitable treatment methods. Methods: The clinical manifestations of one case of long-term fever in adult EBV-HLH were reported, the results of pathology and treatment were examined, and the literature was discussed and analyzed. Results: The patients with EBV-HLH had recurrent fever with pancytopenia, hepatosplenomegaly and lymphadenopathy as the first symptom, followed by ascites. The course of the disease was 16 months. Pathomorphology and immunohistochemistry suggested lymphoproliferative diseases- T-cell lymphoma to be investigated, EBV (+). Remission was achieved with VP-16 combined with EBV-CTL treatment. Conclusion: The rare adult EBV-HLH should pay attention to the differential diagnosis of lymphoma, and actively and rationally treat the life-saving of patients.