期刊
LEUKEMIA & LYMPHOMA
卷 52, 期 1, 页码 15-23出版社
INFORMA HEALTHCARE
DOI: 10.3109/10428194.2010.530362
关键词
Hairy cell leukemia; cladribine; IGH; immunogenetics; prognosis
资金
- Istituto Toscano Tumori, Italy
- Siena-AIL (Associazione Italiana contro le Leucemie, Linfomi e Mielomi, Siena Section)
- Hairy Cell Leukemia Research Foundation (Illinois, USA)
- Associazione Italiana per la Ricerca contro il Cancro (AIRC), Milan, Italy
- Fondazione MPS (Siena, Italy)
The B cell receptor (BCR) is the functional distinguishing unit that defines any B cell. Immunoglobulin gene (IG) status is preserved in the neoplastic B cell clone and can provide an indicator of the maturation stage reached by the B cell prior to transformation. In hairy cell leukemia (HCL), several pieces of data from IG analysis provide clear hints regarding the cell of origin and the ongoing selective interactions of the tumor BCR with environmental stimuli. HCLs have variable levels of IG somatic mutations, and continue somatic mutations at low levels as well as IG class switching after transformation. More recent data also show the occurrence of selective events in the light chain of the BCR, suggesting a dominant role for IG status in the pathogenesis of HCL. Moreover, it has recently emerged that an unmutated status of the HCL IG can be associated with failure to respond to cladribine, genetic abnormalities indicative of poor outcome, and aggressive disease. These observations suggest that IG analysis may have biological and prognostic relevance in HCL and merits further characterization.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据