非特异型外周T细胞淋巴瘤1例并文献复习

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

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。

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.