Chen Bojiang, Gao Jun, Tang Yuan, Zhang Shangfu, Li Weimin, Zeng Jing
Department of Respiratory Medicine, West China Hospital of Sichuan University, Chengdu 610041, China.
Zhongguo Fei Ai Za Zhi. 2011 May;14(5):446-51. doi: 10.3779/j.issn.1009-3419.2011.05.12.
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.
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.
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.
Since there were no specific clinical features for patients of PMZL-MALT, histopathological examination was the only effective means to confirm the diagnosis.
黏膜相关淋巴组织肺边缘区B细胞淋巴瘤（PMZL - MALT）作为一种罕见疾病，常被误诊。本研究旨在总结该疾病的临床和病理特征，提高医生的认识。
回顾性分析2008年11月至2010年11月在华西医院确诊的7例PMZL - MALT患者（女性5例，男性2例），包括其症状、影像学表现、病理检查、治疗及预后。
患者的中位年龄为62岁（范围34 - 79岁）。6例患者有咳嗽、咳痰症状。肺部实变是最常见的表现，CT检查常误诊为肺炎。6例患者通过纤维支气管镜获得病理诊断，其余1例通过经皮肺穿刺活检确诊。7例患者免疫组化结果显示CD20(+)、CD79a(+)，而CD3ε(-)、CD5(-)、CyclinD1(-)、CD10(-)、Bcl - 2(-)和CD30(-)。此外，Ki - 67的表达低于10%。进一步的PCR分析显示6例患者组织中有免疫球蛋白重链基因重排的证据。根据疾病部位和患者意愿，与2例仅接受对症治疗的患者相比，其他5例接受了化疗。
由于PMZL - MALT患者没有特异性临床特征，组织病理学检查是确诊的唯一有效手段。
