Background and objective: As a rare disease, pulmonary marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (PMZL-MALT), is often misdiagnosed. The aim of this study is to summarize the clinical and pathological features of this disease and improve the awareness of doctors.
Methods: Seven cases (female 5, male 2) diagnosed of PMZL-MALT in West China Hospital between November 2008 and November 2010, were analyzed retrospectively, including their symptoms, radiological findings, pathological examinations, treatment and prognosis.
Results: The median age of the patients were 62 years old (range 34-79 years). Six patients suffered from cough and sputum. Pulmonary consolidation was the most frequent manifestation, leading a misdiagnosis of pneumonia with CT examinations. Pathological diagnosis was obtained via fiberoptic bronchoscopy in six patients and percutaneous pulmonary biopsy for the rest one. In the seven cases, immunohistochemical results showed CD20(+), CD79a(+), while CD3 epsilon(-), CD5(-), CyclinD1(-), CD10(-), Bcl-2(-) and CD30(-). Additionally, the expression of Ki-67 was below 10%. Further PCR analysis showed evidence of immunoglobulin heavy chain gene rearrangement in tissues from six subjects. Based on the disease location and patients' wishes, compared with two cases just receiving symptomatic treatments, the other five ones took in chemotherapies.
Conclusion: Since there were no specific clinical features for patients of PMZL-MALT, histopathological examination was the only effective means to confirm the diagnosis.
背景与目的: 肺粘膜相关淋巴组织边缘区B细胞淋巴瘤（pulmonary marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue, PMZL-MALT）极少见，易误诊。本文旨在总结该病特征，以提高临床医生的认识水平。
方法: 回顾性分析四川大学华西医院2008年11月-2010年11月确诊的7例PMZL-MALT患者的临床病理资料。
结果: 女性5例，男性2例；中位年龄62岁（37岁-74岁）。以咳嗽、咳痰为主要症状（6例）；CT多表现为肺部实变影（6例），误诊为肺炎。纤维支气管镜是主要的侵入性检查方法（6例）；7例患者肿瘤细胞免疫表型均为CD20和CD79a细胞膜弥漫强阳性表达，CD3ε、CD5、CyclinD1、CD10、Bcl-2和CD30不表达，Ki-67指数 < 10%；6例IgH基因重排分析可见克隆性条带。3例患者予以COP方案（环磷酰胺、长春新碱、泼尼松）化疗，其中1例加利妥昔单抗（RCOP）；1例予以CHOP方案（环磷酰胺、阿霉素、长春新碱、泼尼松）化疗；1例因糖尿病血糖控制不佳予以CTX（环磷酰胺）化疗；2例拒绝化疗。随访至2010年12月2日，仅1例既往有慢性阻塞性肺疾病史且未化疗者在确诊12个月后因呼吸衰竭死亡，其余患者的病情控制可。
结论: PMZL-MALT患者的临床表现无特异性，组织病理学检查是确诊的唯一手段，患者的预后较好。