脾B细胞边缘区淋巴瘤的临床病理观察

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);bcl02+(6/6),IgD+(2/4),CD5+(1/4),CD43-(516),cyclin D1-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.

Clinicopathologic study of splenic marginal zone B-cell lymphoma

Objective To study the clinicopathologie 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, lgH 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-pesitive (8/8), IgD-positive in 2 of the 4 cases (2/4), CD5- positive in 1 of the 4 cases(1/4), 6 of the 6 cases were bcl-2-positive,cyclin D1-negafive and bcl-6/CD10- negative, CD43-negafive 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 fludarahine-based chemotherapy helps to achieve complete remission. In general, the prognosis of this lymphoma type is good. The lymphoma cells predominantly grow in micronodular pattern, with atypical monocytoid B cells rimming around the small B cells, which aggregates in the center. The differential diagnosis includes other small B-cell lymphornas and lymphoid hyperplasis of spleen.