Splenic red pulp lymphoma with numerous basophilic villous lymphocytes: a distinct clinicopathologic and molecular entity?
Splenic red pulp lymphoma with numerous basophilic villous lymphocytes: a distinct clinicopathologic and molecular entity?
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DOI：
10.1182/blood-2007-07-098848
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发表时间：
2008-02-15
期刊：
影响因子：
20.3
通讯作者：
Felman, Pascale
中科院分区：
文献类型：
--
作者：
Traverse-Glehen, Alexandra;Baseggio, Lucile;Felman, Pascale
淋巴瘤患者中循环绒毛淋巴细胞（VL）的存在通常表明脾边缘区B细胞淋巴瘤（SMZL），即使偶尔可以在其他小B细胞淋巴瘤中发现VL。然而，这些细胞被描述得不多，并且通常缺乏详细的细胞学特征。我们在大量的脾淋巴瘤伴VL中发现了大量嗜碱性VL的淋巴瘤病例，并对37例具有这种特殊细胞学的病例进行了分析。患者，主要是老年男性，表现为中度淋巴细胞增多和脾肿大，无全血细胞减少。单克隆B细胞表达IgM + D、IgM + G、IgM或IgG，以及CD 76和CD 11 c，经常表达CD 103，很少表达CD 123。脾脏切片很特殊，萎缩的白色髓和充血的红髓中的单形弥漫性淋巴瘤浸润。骨髓浸润呈间质和窦内浸润，无广泛纤维化。细胞遗传学分析显示，经常缺乏克隆畸变（68%）。IgH突变占79%，以V（H）3和V（H）4基因家族为主。这些结果以及临床演变表明，这些淋巴瘤病例代表了一个不同于SMZL的同质组，并使人联想到毛细胞白血病变体，可能对应于一个单独的淋巴瘤实体。
The presence of circulating villous lymphocytes (VLs) in lymphoma patients usually points to splenic marginal zone B-cell lymphoma (SMZL), even if the VLs can be found occasionally in other small B-cell lymphomas. However, those cells are variably described, and detailed cytologic characterization is often lacking. We identified lymphoma cases with numerous basophilic VLs among the large group of splenic lymphoma with VLs, and for further delineation, 37 cases with this particular cytology were analyzed. Patients, predominantly older men, presented with moderate lymphocytosis and splenomegaly without pancytopenia. The monoclonal B cells expressed IgM + D, IgM + G, IgM or IgG, as well as CD76 and CD11c, frequently CD103, and rarely CD123. Spleen sections were peculiar, with atrophic white pulp and a monomorphic diffuse lymphoma infiltration in a congested red pulp. Bone marrow infiltration was interstitial and intrasinusoidal without extensive fibrosis. Cytogenetic analysis showed a frequent absence of clonal aberrations (68%). Most cases (79%) were IgH mutated, with an overrepresentation Of V(H)3 and V(H)4 gene families. These results, as well as the clinical evolution,, Show that those lymphoma cases represent a homogeneous group distinct from SMZL and reminiscent of hairy cell leukemia variant, perhaps corresponding to a separate lymphoma entity.