Dogan Ahmet, Isaacson Peter G
Department of Histopathology, University, College London, London, United Kingdom.
Semin Diagn Pathol. 2003 May;20(2):121-7. doi: 10.1016/s0740-2570(03)00012-1.
Splenic marginal zone lymphoma (SMZL) is an indolent lymphoproliferative disease accounting for approximately 1% of all lymphomas. SMZL presents with marked splenomegaly, and often accompanied by circulating atypical 'villous lymphocytes' and is also known as splenic lymphoma with villous lymphocytes. Histologically, the spleen in SMZL is characterised by a nodular infiltrate based on pre-existing white pulp but also involving the red pulp. Within the white pulp, the infiltrate has a biphasic morphology comprising an inner zone of small lymphocytes and a peripheral (marginal) zone of larger lymphoid cells. Usually the splenic lymph nodes and bone marrow are also involved by a vaguely nodular infiltrate of similar nature. Immunophenotypically, the tumor cells has a mature B-cell phenotype and frequently express IgM and IgD but typically lack CD5, CD23, CD43, CD10, Bcl-6 and cyclin D1. Analysis of immunoglobulin heavy-chain gene variable regions suggest that some cases of SMZL arise form postfollicular B cells but others from naïve B cells. Genetic studies have shown abnormalities of a number of chromosomes however 7q31-32 allelic loss appears to be characteristic. Histological differential diagnosis include a number of entities such as lymphoid hyperplasias, other marginal zone lymphomas, mantle cell lymphoma, follicular lymphoma, and B-CLL.
脾边缘区淋巴瘤（SMZL）是一种惰性淋巴细胞增殖性疾病，约占所有淋巴瘤的1%。SMZL表现为明显的脾肿大，常伴有循环中的非典型“绒毛状淋巴细胞”，也被称为伴有绒毛状淋巴细胞的脾淋巴瘤。组织学上，SMZL的脾脏特征为基于原有白髓的结节状浸润，也累及红髓。在白髓内，浸润具有双相形态，包括小淋巴细胞的内区和较大淋巴样细胞的外周（边缘）区。通常脾门淋巴结和骨髓也会被类似性质的模糊结节状浸润累及。免疫表型上，肿瘤细胞具有成熟B细胞表型，常表达IgM和IgD，但通常缺乏CD5、CD23、CD43、CD10、Bcl-6和细胞周期蛋白D1。免疫球蛋白重链基因可变区分析表明，部分SMZL病例起源于滤泡后B细胞，而其他病例起源于幼稚B细胞。遗传学研究显示多个染色体存在异常，然而7q31-32等位基因缺失似乎具有特征性。组织学鉴别诊断包括多种疾病，如淋巴组织增生、其他边缘区淋巴瘤、套细胞淋巴瘤、滤泡性淋巴瘤和B细胞慢性淋巴细胞白血病。
