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一般人群中低计数 CLL 样 MBL 会持续存在,尽管具有与 CLL 相同的细胞遗传学异常,但不会出现临床进展。

General population low-count CLL-like MBL persists over time without clinical progression, although carrying the same cytogenetic abnormalities of CLL.

机构信息

Laboratory of B cell Neoplasia, Division of Molecular Oncology, Istituto Scientifico San Raffaele, Via Olgettina 60, Milan, Italy.

出版信息

Blood. 2011 Dec 15;118(25):6618-25. doi: 10.1182/blood-2011-05-357251. Epub 2011 Aug 29.

Abstract

Monoclonal B-cell lymphocytosis (MBL) is classified as chronic lymphocytic leukemia (CLL)-like, atypical CLL, and CD5(-) MBL. The number of B cells per microliter divides CLL-like MBL into MBL associated with lymphocytosis (usually detected in a clinical setting) and low-count MBL detected in the general population (usually identified during population screening). After a median follow-up of 34 months we reevaluated 76 low-count MBLs with 5-color flow cytometry: 90% of CLL-like MBL but only 44.4% atypical CLL and 66.7% CD5(-) MBL persisted over time. Population-screening CLL-like MBL had no relevant cell count change, and none developed an overt leukemia. In 50% of the cases FISH showed CLL-related chromosomal abnormalities, including monoallelic or biallelic 13q deletions (43.8%), trisomy 12 (1 case), and 17p deletions (2 cases). The analysis of the T-cell receptor β (TRBV) chains repertoire showed the presence of monoclonal T-cell clones, especially among CD4(high)CD8(low), CD8(high)CD4(low) T cells. TRBV2 and TRBV8 were the most frequently expressed genes. This study indicates that (1) the risk of progression into CLL for low-count population-screening CLL-like MBL is exceedingly rare and definitely lower than that of clinical MBL and (2) chromosomal abnormalities occur early in the natural history and are possibly associated with the appearance of the typical phenotype.

摘要

单克隆 B 细胞淋巴增生症 (MBL) 分为慢性淋巴细胞白血病 (CLL) 样、不典型 CLL 和 CD5(-)MBL。每微升 B 细胞数量将 CLL 样 MBL 分为与淋巴增生相关的 MBL(通常在临床环境中检测到)和在普通人群中检测到的低计数 MBL(通常在人群筛查中识别)。在中位随访 34 个月后,我们用 5 色流式细胞术重新评估了 76 例低计数 MBL:90%的 CLL 样 MBL 但只有 44.4%的不典型 CLL 和 66.7%的 CD5(-)MBL 随时间推移而持续存在。人群筛查 CLL 样 MBL 细胞计数无明显变化,无一例发展为明显白血病。50%的病例 FISH 显示 CLL 相关染色体异常,包括单等位基因或双等位基因 13q 缺失(43.8%)、12 三体(1 例)和 17p 缺失(2 例)。T 细胞受体 β (TRBV) 链库分析显示存在单克隆 T 细胞克隆,尤其是在 CD4(high)CD8(low)、CD8(high)CD4(low)T 细胞中。TRBV2 和 TRBV8 是最常表达的基因。本研究表明:(1)低计数人群筛查 CLL 样 MBL 进展为 CLL 的风险非常罕见,肯定低于临床 MBL;(2)染色体异常在自然史早期发生,可能与典型表型的出现有关。

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