Huh Yang O, Abruzzo Lynne V, Rassidakis George Z, Parry-Jones Nilima, Schlette Ellen, Brito-Bapabulle Vasantha, Matutes Estella, Wotherspoon Andrew, Keating Michael J, Medeiros L Jeffrey, Catovsky Daniel
Department of Hematopathology, MD Anderson Cancer Centre, The University of Texas, Houston, TX 77030, USA.
Br J Haematol. 2007 Jan;136(2):220-8. doi: 10.1111/j.1365-2141.2006.06416.x. Epub 2006 Nov 27.
The t(14;19)(q32;q13), involving the BCL3 locus at chromosome 19q13 and the immunoglobulin heavy chain gene at 14q32, is a rare recurrent cytogenetic abnormality identified in B-cell neoplasms, most of which have been classified as chronic lymphocytic leukaemia (CLL) in the literature. We describe the clinicopathological, immunophenotypic and cytogenetic findings in seven patients with B-cell neoplasms associated with t(14;19)(q32;q13). There were five men and two women, with a median age of 48 years (range 33-68). All had absolute lymphocytosis, six had lymphadenopathy, and one had splenomegaly. Lymphocytes in blood and bone marrow aspirate smears were predominantly small and cytologically atypical. Flow cytometric immunophenotyping showed an atypical immunophenotype with low CLL scores. The growth pattern in bone marrow biopsy specimens was interstitial to diffuse; immunohistochemical stains were positive for bcl3 and negative for cyclin D1. Lymph node biopsy specimens of two patients revealed total architectural effacement by neoplasm with proliferation centres. In addition to t(14;19), cytogenetic studies demonstrated trisomy 12 in five patients. These results suggest that B-cell neoplasms with the t(14;19)(q32;q13) present frequently as leukaemia composed of small B-lymphocytes and share many features with CLL. However, these neoplasms also differ from CLL cytologically and in their immunophenotype.
涉及19号染色体q13处的BCL3基因座和14号染色体q32处的免疫球蛋白重链基因的t(14;19)(q32;q13)，是在B细胞肿瘤中发现的一种罕见的复发性细胞遗传学异常，在文献中大多数此类肿瘤被归类为慢性淋巴细胞白血病（CLL）。我们描述了7例与t(14;19)(q32;q13)相关的B细胞肿瘤患者的临床病理、免疫表型和细胞遗传学特征。患者中男性5例，女性2例，中位年龄48岁（范围33 - 68岁）。所有患者均有绝对淋巴细胞增多，6例有淋巴结病，1例有脾肿大。血液和骨髓穿刺涂片的淋巴细胞主要为小淋巴细胞且在细胞学上不典型。流式细胞术免疫表型分析显示为非典型免疫表型，CLL评分低。骨髓活检标本的生长模式为间质型至弥漫型；免疫组化染色bcl3阳性，细胞周期蛋白D1阴性。2例患者的淋巴结活检标本显示肿瘤完全破坏组织结构并伴有增殖中心。除t(14;19)外，细胞遗传学研究显示5例患者存在12号染色体三体。这些结果表明，伴有t(14;19)(q32;q13)的B细胞肿瘤常表现为小B淋巴细胞组成的白血病，且与CLL有许多共同特征。然而，这些肿瘤在细胞学和免疫表型上也与CLL不同。
