套细胞淋巴瘤10例临床病理分析

Mantle cell lymphoma: a clinicopathological study of 10 cases

目的探讨套细胞淋巴瘤(MCL)的临床病理学特征、诊断与鉴别诊断及预后。方法对10例MCL的临床资料、病理学形态和免疫组化标记进行回顾性分析,并进行随访。结果 10例中男性7例,女性3例,均为中老年人。肿瘤组织表现为淋巴结基本结构不同程度消失,呈弥漫性或套层性(含有生发中心)或模糊不清的结节性方式增生,可见散在"星空"现象,细胞间小血管清晰可见及伴透明变性轻度硬化。经典(普通)MCL最多见,主要由形态较单一的小至中等大核形不规则的中心细胞样B细胞构成,可呈套区、结节和弥漫性生长,是小B淋巴细胞性恶性淋巴瘤的一种。免疫组化显示10例肿瘤细胞CD20、CD79α、Pax-5、bcl-2、cyclinD1和SOX11均(+),9例CD5(+);Ki-67阳性率为20%~50%。随访3例存活<3年,3例存活3~5年,1例存活>5年(87个月),另3例在随访中。结论 MCL是一类比较少见的兼有惰性及具侵袭性非霍奇金淋巴瘤最差特点的B细胞性淋巴瘤。以老年男性发病为主(男女之比>2∶1),最常累及淋巴结,临床分期高,预后差。熟悉该肿瘤的形态学特点和免疫学表型有助于与其他恶性淋巴瘤的鉴别。

Purpose To discuss the clinical pathological features,diagnosis and differential diagnosis,prognosis of mantle cell lymphoma（ MCL）. Methods 10 cases of MCL were collected,their clinical pathological characteristics and immunophenotypes were also observed,and the prognosis was followed up. Results Of the 10 cases of MCL,7 were men and 3 were women,and they were all of the middle-aged and old people. The performance of the tumor was that the structure of the lymph node disappeared in different degrees,including diffused or dim nodular proliferation. There was phenomenon of scattered starry sky and small blood vessels between cells were clearly visible with hyaline degeneration and mild rigidification. Most of the MCL belonged to the classial type. It was made up of centrocyte-like B cells,whose nuclei were monomorphic,irregular and small to medium in size,including diffused or nodular proliferation. It was one of the small B cell lymphoma. The immunohistochemistry showed positive CD20（ 10 /10）,CD79α（ 10 /10）,Pax-5（ 10 /10）,CD5（ 9 /10）,bcl-2（ 10 /10）,cyclinD1（ 10 /10） and SOX11（ 10 /10）. The Ki-67 was 20%- 50% positive. Follow-up results showed that three people of the ten cases survived less than three years,three people survived three to five years,one person survived more than five years（ 87 months）,and the rest three people were following up. Conclusion MCL is one of the few types of B cells lymphoma,which features both indolent and aggressive non-Hodgkin＇s lymphoma. It is dominated by elderly male patients and lymph nodes are most frequently involved,with higher clinical stages and poorer prognosis. To be familiar with clinical pathological features and immunophenotypes is useful to improve the differential diagnosis with other typs of lymphoma.