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目的探讨原发性肺外周T细胞淋巴瘤的临床病理特征。方法采用形态学观察、免疫组化和基因重排检测方法,对1例原发性肺外周T细胞淋巴瘤进行临床病理分析,并复习文献。结果患者女性,75岁。咳嗽10天伴乏力、盗汗。影像学检查示右肺巨大占位。全身检查无其他部位淋巴瘤或白血病证据。支气管镜黏膜活检发现,支气管黏膜有较多异型淋巴细胞浸润。异型淋巴细胞LCA、CD3、CD45RO(+),CD5、CD4、CD8、CD10、TDT、CD56、granzymeB和TIA-1(-);B细胞标记CD20、PAX-5和髓系标记MPO也呈(-)。T细胞受体基因重排检测示T细胞呈单克隆性增生。病理诊断为原发性肺外周T细胞淋巴瘤。因患者右肺病变弥漫,临床分期IV期,予减量CHOP方案化疗3次,但最终因肺部感染难以控制于发病后2.6个月死亡。结论原发性肺外周T细胞淋巴瘤罕见,在出现其他疾病难以解释的肺部实性占位伴全身症状时应考虑该病,需要与肺原发或转移性癌、其他类型的原发或继发淋巴瘤及肺反应性淋巴组织增生鉴别。
Objective To investigate the clinicopathological features of primary pulmonary peripheral T-cell lymphoma. Methods A case of primary pulmonary peripheral T-cell lymphoma was analyzed by morphological observation, immunohistochemistry and gene rearrangement. Clinicopathological analysis and review of the literature. Results Female patient, 75 years old. Cough 10 days with fatigue, night sweats. Imaging examination showed a huge right lung occupancy. No other body parts of lymphoma or leukemia evidence. Bronchoscopy mucosal biopsy found that bronchial mucosa more atypical lymphocytes infiltration. CD3, CD45RO (+), CD5, CD4, CD8, CD10, TDT, CD56, granzymeB and TIA-1 (-) were also detected in allogeneic lymphocytes; B cell markers CD20, PAX- ). T cell receptor gene rearrangement test showed T cells showed monoclonal hyperplasia. Pathological diagnosis of primary pulmonary peripheral T cell lymphoma. Because of diffuse right lung lesions, clinical stage IV, to reduce the dose of CHOP chemotherapy 3 times, but eventually due to pulmonary infection is difficult to control after the onset of 2.6 months of death. CONCLUSIONS: Primary pulmonary peripheral T-cell lymphoma is rare and should be considered in the presence of solid masses in the lung that are difficult to interpret with other diseases and need to be associated with primary or metastatic lung cancer, other types of primary or Secondary lymphoma and pulmonary reactive lymphoid tissue differentiation.