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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 diagnosis of peripheral T lymphoma with fever as the main manifestation, and to explore its clinical features and treatment methods. Methods: A retrospective analysis of one case of non-specific peripheral T-cell lymphoma diagnosis and treatment process, combined with literature review, in order to improve the diagnosis and treatment of the disease. Results: Patients from the onset to a clear diagnosis of more than 1 years, the course of repeated fever, rash, abnormal liver function. After 2 lymph node biopsy, 1 spleen puncture, confirmed by pathological immunohistochemical analysis. Has given EPOCH, DICE, GemOx and other first-line, second-line treatment programs, and the application of anti-angiogenic drugs Endostar Endo adjuvant therapy, the condition was once stable. After 8 months of diagnosis, the disease progression and hemophagocytic syndrome died. Conclusion: Cases of fever and painless lymphadenopathy are the main manifestations. Serum LDH and β2-microglobulin are significantly elevated. When lymphoma is highly suspected, multiple biopsies should be taken. Peripheral T-cell lymphoma has poor response to chemotherapy with EPOCH regimen. The GemOx regimen containing gemcitabine and platinum can delay the progression of the disease, but the maintenance time is short and the prognosis of lymphoma combined with hemophagocytic syndrome is poor.