脾B细胞边缘区淋巴瘤的临床病理观察  

Clinicopathologic study of splenic marginal zone B-cell lymphoma

作　　者：张彦宁[1] 郑媛媛[1] 周小鸽[1] 张淑红[1] 金妍[1] 谢建兰[1] 陈书媛[1] 石岩[2] 吴丽华[2] 

机构地区：[1]首都医科大学附属北京友谊医院病理科,100050 [2]哈尔滨医科大学第二医院病理科

出　　处：《中华病理学杂志》2009年第4期243-247,共5页Chinese Journal of Pathology

基　　金：国家自然科学基金（30670892）

摘　　要：目的分析和总结脾边缘区B细胞淋巴瘤（SMZL）的临床病理特点、探讨其诊断与鉴别诊断要点。方法对8例原发性SMZL的临床资料行回顾性分析总结、组织切片的形态观察和免疫组织化学EliVision法染色分析，并对部分病例行基因重排克隆性检测，获得4例随访资料。结果8例SMZL的中位年龄为61．5岁（36～75岁），男女比例为1．7：1。患者均因脾大就诊，5例伴血象异常，白细胞和血小板均低于正常，其中2例全血细胞下降。脾切除后3例血象全部或部分恢复正常。3例福达华联合化疗后，2例完全缓解，1例死亡。随访4例的平均生存期21．5个月（6～60个月）。在病理形态上，8例脾脏均呈白髓结节状增生，其中6例由经典的两种细胞组成，其分布表现为结节中央密集而深染的小淋巴细胞，周围为不典型单核细胞样细胞。2例增生结节全部由形态一致的不典型单核细胞样细胞组成。红髓区片状浸润8例。肿瘤细胞CD20＋（8例）；bcl-2＋（6／6），IgD＋（2／4），CD5＋（1／4），CD43-（5／6），cyclinD1-和bcl-6／CD10-（6／6）。核增殖指数〈15％。结论SMZL为惰性淋巴瘤，以脾大伴血象异常为主要临床表现。脾切除治疗有效，FCD化疗可完全缓解，预后较好。病理形态以白髓结节状增生为主，呈不典型单核细胞样细胞形态，大部分标本结节中央见较小密集的淋巴细胞，同时存在红髓区片状浸润。诊断需除外其他小B细胞淋巴瘤和脾白髓增生。Objective To study the clinicopathologic features, diagnosis and differential diagnosis of splenic marginal zone B-cell lymphoma （SMZL）. Methods The clinical data, histologic findings and immunophenotype of 8 SMZL cases were studied. IgH gene rearrangement was performed in 1 case. Follow- up information was available in 4 patients. Results The median age of the patients was 61.5 years （ range ： 36 to 75 years）. The male-to-female ratio was 1.7： 1. All cases presented with massive splenomegaly. Five of six cases had abnormal blood counts ： neutropenia and thrombocytopenia with two of them showing anemia. After splenectomy, the blood counts in 3/3 cases returned to normal levels. Post-operative fludarabine-based chemotherapy was given to 3 patients, two of them achieved complete remission and 1 case died during the course of chemotherapy. The average survival time was 21.5 months （range： 6 to 60 months ）. Histologically, all of the 8 cases showed micronodular white pulp lesions. Six of them exhibited the classic biphasic appearance with central aggregates of small B cells rimmed by a peripheral zone of atypical monocytoid B cells. The remaining 2 cases had a monomorphous appearance, consisting mainly of atypical monocytoid B cells. There was infiltration of tumor cells in the red pulp, sheets in appearance in all 8 cases. Immuo-histochemical staining showed CD20-positive（8/8）, IgD-positive in 2 of the 4 cases （2/4）, CDS- positive in 1 of the 4 cases （ 1/4）, 6 of the 6 cases were bcl-2-positive, cyclin Dl-negative and bcl-6/CD10- negative, CD43-negative in 5 of the 6 cases （5/6）. The proliferation index, as highlighted by Ki-67 immunostaining, was low （ 〈 15% ）. Conclusions SMZL is an indolent B-cell non-Hodgkin lymphoma. The main clinical manifestations are splenomegaly and abnormalities in blood counts. The main modality of treatment is splenectomy. Adjuvant fludarabine-based chemotherapy helps to achieve complete remission. In general, the prognosis of this lymphoma type

关 键 词：脾肿瘤 淋巴瘤 B细胞 免疫表型分型 诊断 鉴别 

分 类 号：R686[医药卫生—骨科学]