皮下脂膜炎样T细胞淋巴瘤合并噬血细胞综合征2例并文献复习  

Subcutaneous panniculitis-like T-cell lymphoma-associated hemophagocytic syndrome:two cases report and literature review

作　　者：裴瑞君 黄文秋[1] 王旖旎[1] 汤然[1] 李硕[1] 魏娜[1] 

机构地区：[1]首都医科大学附属北京友谊医院血液科,北京100050

出　　处：《临床血液学杂志》2014年第5期779-782,共4页Journal of Clinical Hematology

基　　金：北京友谊医院科研启动基金(No:YYQDKT-7)

摘　　要：目的:探讨皮下脂膜炎样T细胞淋巴瘤(SPTCL)合并噬血细胞综合征(HPS)的诊断及治疗。方法:报道2例SPTCL合并HPS患者并进行文献复习。结果:2例患者治疗过程中HPS反复发作,经多次病理检查最终确诊为SPTCL,给予原发病治疗后长期生存。结论:SPTCL合并HPS少见,原发病诊断困难,病情反复。尽早且多次进行淋巴结、骨髓或病变部位组织病理检查以寻找原发病,给予原发病治疗,是控制SPTCL合并HPS的关键。Objective：To investigate the diagnosis and therapy of subcutaneous panniculitis-like T-cell lymphoma（SPTCL）-associated hemophagocytic syndrome（HPS）.Method：Two cases of SPTCL-associated HPS were reported and relative literatures were reviewed.Result：Two patients were diagnosed as HPS exhibited as a recurrent disease course.Diagnoses of SPTCL were finally established after repeated pathological examination.After the treatment of primary diseases,long-term survival was accessible.Conclusion：SPTCL-associated HPS is a rare disorder with a recurrent disease course,and it is difficult to determine the primary disease.To control the disease course,it is crucial to make early and repeated biopsy of lymph node,bone marrow and the lesion and treat primary diseases.

关 键 词：皮下脂膜炎样T细胞淋巴瘤 继发性 噬血细胞综合征 

分 类 号：R733.4[医药卫生—肿瘤]