Department of Medicine, Duke University Medical Center, 1 Trent Drive, Morris Building Room 25153, Durham, NC 27710, USA.
Leukemia. 2010 Jan;24(1):133-40. doi: 10.1038/leu.2009.192. Epub 2009 Oct 15.
Monoclonal B-cell lymphocytosis (MBL) is a preclinical hematologic syndrome characterized by small accumulations of CD5(+) B lymphocytes. Most MBL share phenotypic characteristics with chronic lymphocytic leukemia (CLL). Although some MBL progress to CLL, most MBL have apparently limited potential for progression to CLL, particularly those MBL with normal absolute B-cell counts ('low-count' MBL). Most CLL are monoclonal and it is not known whether MBL are monoclonal or oligoclonal; this is important because it is unclear whether MBL represent indolent CLL or represent a distinct premalignant precursor before the development of CLL. We used flow cytometry analysis and sorting to determine immunophenotypic characteristics, clonality and molecular features of MBL from familial CLL kindreds. Single-cell analysis indicated four of six low-count MBL consisted of two or more unrelated clones; the other two MBL were monoclonal. 87% of low-count MBL clones had mutated immunoglobulin genes, and no immunoglobulin heavy-chain rearrangements of V(H) family 1 were observed. Some MBL were diversified, clonally related populations with evidence of antigen drive. We conclude that although low-count MBL share many phenotypic characteristics with CLL, many MBL are oligoclonal. This supports a model for step-wise development of MBL into CLL.
单克隆 B 细胞淋巴增生症(MBL)是一种临床前血液学综合征,其特征是少量 CD5(+) B 淋巴细胞积聚。大多数 MBL 具有与慢性淋巴细胞白血病(CLL)相似的表型特征。虽然一些 MBL 进展为 CLL,但大多数 MBL 显然进展为 CLL 的潜力有限,特别是那些绝对 B 细胞计数正常的 MBL(“低计数”MBL)。大多数 CLL 是单克隆的,目前尚不清楚 MBL 是单克隆还是寡克隆;这很重要,因为目前尚不清楚 MBL 是惰性 CLL 的代表,还是在发展为 CLL 之前代表一种不同的癌前前体。我们使用流式细胞术分析和分选来确定来自家族性 CLL 家族的 MBL 的免疫表型特征、克隆性和分子特征。单细胞分析表明,六个低计数 MBL 中有四个由两个或多个无关克隆组成;另外两个 MBL 是单克隆的。87%的低计数 MBL 克隆具有突变的免疫球蛋白基因,并且没有观察到 V(H)家族 1 的免疫球蛋白重链重排。一些 MBL 是具有抗原驱动证据的多样化、克隆相关的群体。我们得出结论,尽管低计数 MBL 与 CLL 具有许多表型特征,但许多 MBL 是寡克隆的。这支持了 MBL 逐步发展为 CLL 的模型。