A distinctive subtype of t(14;18)-negative nodal follicular non-Hodgkin lymphoma characterized by a predominantly diffuse growth pattern and deletions in the chromosomal region 1p36
A distinctive subtype of t(14;18)-negative nodal follicular non-Hodgkin lymphoma characterized by a predominantly diffuse growth pattern and deletions in the chromosomal region 1p36
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DOI：
10.1182/blood-2008-07-168682
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发表时间：
2009-01-29
期刊：
影响因子：
20.3
通讯作者：
Ott, German
中科院分区：
文献类型：
--
作者：
Katzenberger, Tiemo;Kalla, Joerg;Ott, German
滤泡性淋巴瘤(FL)是一种形态和遗传学特征良好的B细胞性非霍奇金淋巴瘤，可表现为以滤泡型为主、滤泡型和弥漫型相结合或以弥漫型为主的生长模式。虽然大约85%的FLS携带t(14；18)(q32；q21)易位并持续呈现卵泡生长模式，但主要是弥漫性FLS在表型、分子和临床水平上的特征较差。我们通过免疫组织化学、经典染色体显带分析、荧光原位杂交(FISH)和基因表达谱分析，研究了35例主要弥漫性FL。29例可分析病例中共有28例t(14；18)缺失，29例中27例出现统一的染色体异常，其中1p36缺失。病理形态为1级12例，2级23例，CD10、BCL6、CD23表达频繁的免疫表型与生发中心B细胞表型一致。4例以弥漫性为主的FL的基因表达谱属于典型FL的谱系，具有独特的特异基因特征。值得注意的是，弥漫性FL患者的临床分期较低，腹股沟肿瘤较大但有局限性。这些结果表明，弥漫性FL代表了t(14；18)阴性结节性FL的一种独特亚型，具有统一的基因改变(缺失1p36)和典型的临床特征。(血。2009；113：1053-1061)
Follicular lymphoma (FL) is a morphologically and genetically well-characterized B-cell non-Hodgkin lymphoma that can show predominantly follicular, combined follicular and diffuse, or predominantly diffuse growth patterns. Although approximately 85% of FLs harbor the translocation t(14;18)(q32;q21) and consistently display a follicular growth pattern, predominantly diffuse FLs are less well characterized on the phenotypical, molecular, and clinical level. We studied 35 predominantly diffuse FL by immunohistochemistry, classical chromosome banding analysis, fluorescence in situ hybridization (FISH), and gene expression profiling. A total of 28 of 29 analyzable cases lacked t(14;18), and 27 of 29 cases revealed a unifying chromosomal aberration, a deletion in 1p36. Morphologically, 12 FLs were grade 1 and 23 were grade 2, and the immunophenotype with frequent expression of CD10, BCL6, and CD23 was in line with a germinal center B-cell phenotype. The gene expression profiles of 4 predominantly diffuse FLs fell into the spectrum of typical FL, with a unique enrichment of specific gene signatures. Remarkably, patients with diffuse FL frequently presented with low clinical stage and large but localized inguinal tumors. These results suggest that predominantly diffuse FL represent a distinct subtype of t(14;18)-negative nodal FL with a unifying genetic alteration (deletion of 1p36) and characteristic clinical features. (Blood. 2009;113:1053-1061)