Hernandez J M, Mecucci C, Michaux L, Criel A, Stul M, Meeus P, Wlodarska I, Van Orshoven A, Cassiman J J, De Wolf-Peeters C, Van den Berghe H
Centre for Human Genetics, University of Leuven, Belgium.
Cancer Genet Cytogenet. 1997 Feb;93(2):147-51. doi: 10.1016/s0165-4608(96)00183-5.
Twelve patients with diagnosis of B-cell non-Hodgkin's lymphoma/leukemia and del[7q] were studied for their clinical, cytogenetic, and molecular characteristics. Eleven patients were classified as small cell lymphoma whereas one had a diffuse large cell lymphoma. Lymphoplasmacytic features were observed in six out of eleven small cell lymphomas. Morphologically and immunologically these small cell lymphomas could be classified as chronic lymphocytic leukemia (typical or atypical; 4 cases), marginal zone lymphoma (splenic lymphoma with villous lymphocytes; 1 case), mantle cell lymphoma (2 cases), or nonspecified, non-Hodgkin's lymphoma (4 cases). Eleven of twelve patients presented with peripheral blood and bone marrow involvement. Two of twelve cases showed del[7q] as the sole anomaly. Two different types of deletions were present: ten cases had del(7)(q21q31) and two cases had del(7)(q31q34). Cases that could be molecularly investigated did not show any involvement of BCL2, BCL3, or BCL6, and only one case had BCL1 rearrangement. The data indicate that del(7q) is associated with a subset of mature small B-cell lymphoproliferative disorders of which some but not all show lymphoplasmatic features.
对12例诊断为B细胞非霍奇金淋巴瘤/白血病且存在7号染色体长臂缺失(del[7q])的患者进行了临床、细胞遗传学和分子特征研究。11例患者被归类为小细胞淋巴瘤,1例为弥漫性大B细胞淋巴瘤。11例小细胞淋巴瘤中有6例具有淋巴浆细胞特征。从形态学和免疫表型上看,这些小细胞淋巴瘤可分类为慢性淋巴细胞白血病(典型或非典型,4例)、边缘区淋巴瘤(脾淋巴瘤伴绒毛状淋巴细胞,1例)、套细胞淋巴瘤(2例)或未明确分类的非霍奇金淋巴瘤(4例)。12例患者中有11例出现外周血和骨髓受累。12例中有2例仅表现为7号染色体长臂缺失这一异常。存在两种不同类型的缺失:10例为del(7)(q21q31),2例为del(7)(q31q34)。能进行分子检测的病例未显示BCL2、BCL3或BCL6有任何受累情况,仅1例存在BCL1重排。数据表明,7号染色体长臂缺失与成熟小B细胞淋巴增殖性疾病的一个亚组相关,其中部分(而非全部)表现出淋巴浆细胞特征。