肺原发性黏膜相关淋巴组织淋巴瘤临床特征分析

Clinical manifestations analysis of patients diagnosed with primary pulmonary mucosa-associated lymphoid tissue lymphoma

目的探讨分析肺原发性黏膜相关淋巴组织（MALT）淋巴瘤的临床特征。方法回顾性分析2007年5月至2017年5月于复旦大学附属中山医院住院并经病理确诊为肺MALT淋巴瘤的14例患者的临床特点、病理特征、诊断、治疗及预后。结果病理取材中4例通过电视辅助胸腔镜手术（VATS），2例通过经皮穿刺肺活检，8例经支气管镜肺穿刺活检。病理检查结果：瘤细胞主要由中心细胞样淋巴细胞、小淋巴细胞样瘤细胞、单核样B细胞组成；瘤细胞均表达B细胞相关抗原。中心细胞样细胞型10例，单核细胞型和小淋巴细胞型各2例。4例行IgH基因重排检测，其中3例出现单克隆条带。6例行双色荧光原位杂交（FISH）MALT1基因分离探针检测，其中3例阳性。14例患者单纯化疗8例，手术切除5例；手术病例术后化疗2例，1例放弃治疗；随访12例，随访时间1-9年，复发2例，死亡2例。结论肺原发性MALT淋巴瘤临床表现缺乏特异性，确诊主要依据病变部位病理组织活检、免疫组织化学检测、IgH克隆性检测以及FISH检测MALT1l基因。

Objective To analyze the clinical features of pulmonary mucosa-associated lymphoid tissue （MALT） lymphoma. Methods A total of 14 cases diagnosed with primary pulmonary MALT lymphoma were collected from May 2007 to May 2017 in Zhongshan Hospital, Fudan University. The clinical features, pathological characteristics, diagnosis, treatment and prognosis were retrospectively analyzed. Results All 14 cases were pathologically diagnosed with primary pulmonary MALT lymphoma. The biopsy tissues were obtained through the Video-assisted Thoracoscopic Surgery （VATS） （4 cases）, percutaneous puncture （2 cases）, and bronchoscopy （8 cases）. Cell types of these tumors were centrocyte-like cells （10 cases）, lymphocytoid cells （2 cases）, and monoeytoid B cells （2 cases）. The B cell clonality was detected by IgH cloning test in 4 cases and 3 of them were demonstrated with monoclonal strips. MALT1 breakup gene was positive in 3 out of 6 examined cases using fluorescence in situ hybridization （FISH）. As for the treatment, 8 patients underwent chemotherapy, 5 patients underwent surgical resection and 1 patient abandoned treatment. Twelve patients were followed up to 9 years. The tumor recurrence occurred in 2 patients and resulted their death. Conclusions The clinical manifestations of primary pulmonary MALT lymphoma are lack of specificity. The pathology, immunohistochemistry, IgH cloning test and MALT1 breakup gene tested by FISH are the criteria for diagnosis.