11q畸变的高级别b细胞淋巴瘤:一项单中心研究

Shoki Yamada, Yuka Oka, Moe Muramatsu, Yuko Hashimoto
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摘要

高级别b细胞淋巴瘤伴11q畸变(High-grade b cell lymphoma with 11q aberrations, HGBL-11q)在世界卫生组织第五版《造血淋巴组织肿瘤分类》中首次被归类为高级别成熟b细胞肿瘤。HGBL-11q在形态学和免疫组织化学上与伯基特淋巴瘤(BL)或HGBL相似;其特点是11q23.2-11q23.3区域增加,11q24.1-qter区域减少,但缺乏MYC易位。HGBL-11q是一种罕见的肿瘤,其在日本的确切发病率尚不清楚。在这项研究中,我们对113例生发中心b细胞(GCB)型侵袭性b细胞淋巴瘤(BCLs)进行了分类,分为BL、高级别(HG)和大细胞(LC)形态。我们用荧光原位杂交(FISH)鉴定11q像差。9例患者有11q畸变(7.96%,9/113),其中HGBL-11q 6例。年龄8 ~ 87岁,均为男性。14例HG形态学患者中有6例被诊断为HGBL-11q(6/14, 42.9%)。HGBL-11q主要发生在儿童和年轻人中,但也发生在中年和老年人中。没有MYC易位的HG形态学患者,无论年龄大小,都应进行11q异常的FISH检查。然而,HGBL-11q的发病机制、临床表现和预后尚不清楚。在日常实践中积累准确诊断出HGBL-11q的病例,以及准确详细的HGBL-11q数据,将有助于进一步了解11q畸变。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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High-grade B-cell lymphoma with 11q aberrations: A single-center study.

High-grade B-cell lymphoma with 11q aberrations (HGBL-11q) has been classified for the first time as a high-grade mature B-cell neoplasm according to the 5th edition of the World Health Organization Classification of Tumors of Hematopoietic and Lymphoid Tissues. HGBL-11q is morphologically and immunohistochemically similar to Burkitt lymphoma (BL) or HGBL; it is characterized by gain in the 11q23.2-11q23.3 region and loss in the 11q24.1-qter region but it lacks MYC translocation. HGBL-11q is a rare tumor, and its exact frequency in Japan remains unclear. In this study, we classified 113 Germinal center B-cell (GCB) type aggressive B-cell lymphomas (BCLs), which were divided into BL, high-grade (HG), and large cell (LC) morphologies. We performed fluorescence in situ hybridization (FISH) to identify 11q aberrations. Nine patients had 11q aberrations (7.96%, 9/113), including six HGBL-11q. The age range was from 8 to 87 years, and all were male. Six out of 14 patients with HG morphology were diagnosed with HGBL-11q (6/14, 42.9%). HGBL-11q has been found to occur primarily in children and young adults but also in middle-aged and older adults. Patients with HG morphology without MYC translocation should undergo FISH for 11q aberrations regardless of age. However, the pathogenesis, clinical findings, and prognosis of HGBL-11q remain unclear. The accumulation of cases with an accurate HGBL-11q diagnosis in daily practice and accurate and detailed data on HGBL-11q will contribute to further understanding of 11q aberrations.

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CiteScore
2.00
自引率
6.70%
发文量
25
审稿时长
11 weeks
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