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机构地区:[1]首都医科大学附属复兴医院病理科,北京100038 [2]首都医科大学附属复兴医院血液科,北京100038
出 处:《白血病.淋巴瘤》2009年第3期146-148,共3页Journal of Leukemia & Lymphoma
摘 要:目的探讨脾边缘区淋巴瘤(SMZL)的临床及病理学特征、诊断、治疗及预后。方法详细报道1例随访16年的SMZL患者的临床表现、治疗效果及肿瘤原发灶和转移灶的组织形态学特点,并复习相关文献。结果SMZL临床以脾大为特征,常累及脾门淋巴结、外周血及骨髓,可发生结内及结外转移。形态学上肿瘤细胞小到中等大小,染色质较疏松,免疫组织化学肿瘤细胞sIgM(+)、CD20^+、CD79a^+和bcl-2(+);CD3^-、CD5^-、CD10^-、CD21^-、CD43^-和CyclinD1(-)。结论SMZL是一种少见的原发于脾脏的低度恶性B细胞淋巴瘤,起病潜隐,进展缓慢,但可向大B细胞淋巴瘤转化。脾切除和化疗有一定疗效,但不易治愈。Objective To study the clinical and pathologic features, diagnosis, treatment and prognosis of splenic marginal zone lymphoma (SMZL). Methods We reported the detailed clinical and pathologic presentation of a patient with SMZL, and monitored fellow-up to 16 years. Results Patients present with splenomegaly. SMZL usually involve bone marrow, peripheral blood and hilar splenic lympha nodes. Peripheral lymph nodes and nonhematolymphoid organs can be involved as well, but to a lesser extent. Microscopically, tumor cells are small to medium in size, with dispersed chromation. Tumor cells are sIgM(+), CD20^+, CD79^+, bcl-2(+), CD3^-, CD5^-, CD10^-, CD23^- and CyclinD1(-). Conclusion SMZL is a rare low-grade B-cell lymphoma with an indolent clinical course. Splenectomy and chemotherapy are effective treatment but SMZL cannot be completelv cured and may transform to large B-cell lvmphoma at last.
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