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伴有伯基特样细胞的非霍奇金淋巴瘤与c-Myc扩增及预后不良相关。

Non-Hodgkin's lymphomas with Burkitt-like cells are associated with c-Myc amplification and poor prognosis.

作者信息

Mossafa H, Damotte D, Jenabian A, Delarue R, Vincenneau A, Amouroux I, Jeandel R, Khoury E, Martelli J M, Samson T, Tapia S, Flandrin G, Troussard X

机构信息

Département Génétique, Laboratoire Pasteur Cerba, Cergy Pontoise, France.

出版信息

Leuk Lymphoma. 2006 Sep;47(9):1885-93. doi: 10.1080/10428190600687547.

Abstract

Out of 344 patients with newly diagnosed non-Hodgkin's lymphoma (NHL), this study identified 16 patients presenting Burkitt-like cells (BLCs) after cytological and/or histological review. Conventional cytogenetic analysis showed at diagnosis complex chromosomal abnormalities in 13 cases and a normal karyotype in three cases. However, neither t(8;14)(q24;q32) nor the variants t(2;8)(p12;q24) or t(8;22)(q24;q11) was detected. FISH studies showed c-MYC amplification in all cases with four to more than seven copies in 10 - 77% metaphase or inter-phase cells. This study did not observe any gene fusion signal for c-MYC/IgH excluding a t(8;14) translocation and partial tri or polysomy of chromosome 8. It also excluded in that cases a break apart for the c-MYC locus. This study also never detected IgL/c-MYC, IgK/c-MYC or X-c-MYC. The BLCs were present whatever the lymphoma sub-type: follicular lymphoma (FL) was diagnosed in six out of 16 patients, mantle cell lymphoma (MCL) in four out of 16 patients, marginal zone lymphoma (MZL) in two out of 16 patients and diffuse large B-cell lymphomas (DLBCL) in three out of 16 patients. One additional patient presented a T-cell lymphoma. The clinical course was aggressive with a poor prognosis, as death occurred in nine patients, within 6 months after diagnosis for eight of them. These data could suggest a sub-group of NHL patients (15 B-NHL, 1 T-NHL) have been identified with a poor prognosis characterized by the association of Burkitt-like cells and c-MYC amplification without t(8;14)(q24;q32) or its variants. The possibility that this profile may represent a distinct morphologic NHL sub-set remains to be determined on a large cohort of patients.

摘要

在344例新诊断的非霍奇金淋巴瘤(NHL)患者中,本研究经细胞学和/或组织学检查后确定了16例出现伯基特样细胞(BLCs)的患者。常规细胞遗传学分析显示,诊断时13例患者存在复杂的染色体异常,3例患者核型正常。然而,未检测到t(8;14)(q24;q32) 及其变异体t(2;8)(p12;q24) 或t(8;22)(q24;q11)。荧光原位杂交(FISH)研究显示,所有病例均存在c-MYC扩增,在10%-77%的中期或间期细胞中有4至7个以上拷贝。本研究未观察到c-MYC/IgH的任何基因融合信号,排除了t(8;14)易位以及8号染色体部分三体或多体。该研究还排除了这些病例中c-MYC基因座的断裂分离情况。本研究也从未检测到IgL/c-MYC、IgK/c-MYC或X-c-MYC。无论淋巴瘤亚型如何,均存在BLCs:16例患者中有6例诊断为滤泡性淋巴瘤(FL),16例患者中有4例为套细胞淋巴瘤(MCL),16例患者中有2例为边缘区淋巴瘤(MZL),16例患者中有3例为弥漫性大B细胞淋巴瘤(DLBCL)。另外1例患者为T细胞淋巴瘤。临床病程呈侵袭性,预后较差,9例患者死亡,其中8例在诊断后6个月内死亡。这些数据表明,已识别出一组预后不良的NHL患者(15例B-NHL,1例T-NHL),其特征为伯基特样细胞与c-MYC扩增相关,但无t(8;14)(q24;q32) 或其变异体。这种特征是否代表一种独特的形态学NHL亚组,仍有待在大量患者队列中确定。

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