非特异型外周T细胞淋巴瘤1例并文献复习

叶进; 王景文; 宁丰; 杨磊

doi:10.13201/j.issn.1004-2806.2012.02.006

非特异型外周T细胞淋巴瘤1例并文献复习

- 首都医科大学附属北京同仁医院血液科(北京, 100730)

详细信息

通讯作者: 王景文,E-mail:trwangjw@gmail.com

中图分类号: R733.4

收稿日期: 2011-07-24

Peripheral T-cell lymphoma unspecified:a case report and review of literatures

- Department of Hemotology, Beijing Tongren Hospital, Capital Medical University, Beijing, 100730, China

More Information

Corresponding author: WANG Jingwen, E-mail: trwangjw@gmail.com

Received Date: 24 July 2011

摘要

摘要: 目的:提高以发热为主要表现的外周T淋巴瘤的诊断水平,并探讨其临床特点及治疗方法。方法:回顾性分析1例非特异型外周T细胞淋巴瘤诊治过程,结合文献复习,以期提高该病的诊治水平。结果:患者从发病到明确诊断1年余,病程中反复出现发热、皮疹、肝功能异常。经过2次淋巴结活检、1次脾脏穿刺,经病理免疫组织化学分析得以确诊。先后给予EPOCH、DICE、GemOx等一线、二线治疗方案,并应用抗血管新生药物血管内皮抑制素恩度辅助治疗,病情曾一度稳定。确诊8个月后,病情进展并发噬血细胞综合征死亡。结论:对于以发热、无痛性淋巴结肿大为主要表现的病例,同时伴有血清LDH、β2-微球蛋白明显升高,高度怀疑淋巴瘤时,应反复多次、多部位取活检。外周T细胞淋巴瘤对EPOCH方案化疗反应差,含有吉西他滨和铂类的GemOx方案可延缓病情进展,但维持时间短,淋巴瘤并发噬血细胞综合征预后差。
- 淋巴瘤,T细胞 /
- 诊断 /
- 治疗
Abstract: Objective:To improve the diagnostic level in peripheral T-cell lymphoma unspecified(PTCL-U)with unknown-reason fever as main clinical feature,and to explore the clinical characteristics and therapeutic method. Method: A case of PTCL-U was reported. The diagnostic and treatment course were retrospectively analyzed. The related literatures were reviewed for increasing recognition of the disease. Result:After suffering recurrent fever,rash and abnormal liver function for one year,the patient experienced biopsy of lymph node twice and aspirate of spleen once,she was diagnosed as peripheral T-cell lymphoma unspecified eventually by pathological immunohistochemistry. She received first-line and second-line regimens such as EPOCH,DICE,and GemOx chemotherapy. She also received anti angiogenesis agent Endstar. The disease was controlled for a time. But 8 months later,the primary disease became progressive and was complicated with hemophagocytic syndrome. Conclusion:If a patient presents with long term unknown origin fever,painless lymph node enlargement accompanied by serum LDH increasing,biopsy should be done repeatedly to identify the suspected lymphoma. The prognosis of PTCL-U is poor. In our experience,the effect of EPOCH regimen is limited,GemOx is effective in pretreated PTCL-U patient and can delay the progression of lymphoma,which offers an advantage over conventional chemotherapy.But the duration of response is of short time. The prognosis of PTCL-U will be worse if accompanied with hemophagocytic syndrome.
- peripheral T-cell lymphoma unspecified /
- diagnosis /
- treatment

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参考文献(12)

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