以噬血细胞综合征为首发表现的非霍奇金淋巴瘤二例并文献复习

Non-Hodgkin lymphoma with hemophagocytic syndrome as the first manifestation：report of two cases and review of literature

目的 探讨以噬血细胞综合征（HPS）为首发表现的非霍奇金淋巴瘤（NHL）的特点.方法 回顾性分析2例以HPS为首发表现的B细胞NHL患者的发病及治疗转归,并复习国内外文献.结果 2例患者均以HLH-94为基础的方案控制发热后,继以R-CHOP（地塞米松替代泼尼松）方案化疗反应良好.通过复习文献发现B细胞淋巴瘤相关噬血细胞综合征（B-LAHS）中主要淋巴瘤类型为弥漫大B细胞淋巴瘤,T细胞淋巴瘤相关噬血细胞综合征（T-LAHS）中淋巴瘤类型分布无显著差异.中国、韩国的T-LAHS发病率明显高于B-LAHS,在西方国家两者发病率无显著差异.T细胞淋巴瘤更易罹患HPS.B-LAHS患者中位年龄明显高于T-LAHS,中位生存时间和总生存率都优于T-LAHS.利妥昔单抗的应用为B-LAHS带来生存优势.结论 以HPS为首发表现的B细胞NHL应用依托泊苷联合R-CHOP方案可较快控制病情,后续疗效好.

Objective To explore the features of non-Hodgkin lymphoma （NHL） with hemophagocytic syndrome （HPS） as the initial manifestation at diagnosis. Methods The morbidity and treatment of two B-cell NHL patients with HPS as the initial manifestation were retrospectively analyzed, and the literature were reviewed at home and abroad. Results Two B-cell NHL patients with HPS were treated with the HLH-94 protocol to control febrile condition. After then, R-CHOP protocol （dexamethasone substituted for prednisone） was used and their results were good. In reviewing the literature, it was felt that the main sub-type of B-cell lymphoma-associated HPS （B-LAHPS） was diffuse large B-cell lymphoma. However, sub-types of T-cell lymphoma-associated HPS （T-LAHPS） had no significant difference. The incidence of T-LAHPS was significantly higher than that of B-LAHPS in China and South Korea, but there was no significant difference in the Western countries. T-cell lymphomas were more vulnerable to HPS. The median age of B-LAHPS patients at diagnosis was significantly higher than that of T-LAHPS patients. The median survival and overall survival time of B-LAHPS patients were longer than those of T-LAHPS patients by virtue of the application of rituximab. Conclusion B-cell NHL with HPS as the initial manifestation treated with the 2004-HLH plus R-CHOP protocol could achieve good outcomes.