Hairy cell leukaemia: biological and clinical overview from immunogenetic insights
Hairy cell leukaemia: biological and clinical overview from immunogenetic insights
Hairy cell leukaemia (HCL) is a rare neoplasm of peripheral B cells which represents a paradox in oncology. Despite its largely unknown origin and behaviour, HCL is one of the few example of dramatic success in the treatment of a malignancy. The recent steps forward to understanding the biology of HCL from immunogenetic and genomic studies have recently provided new insight into diagnosis and prognosis. Several data from immunoglobulin gene (IG) analysis have provided hints regarding the cell of origin and the ongoing selective interactions of the tumour BCR with environmental stimuli. It has also 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 a central role of the tumour B-cell receptor in defining the outcome of HCL and that that IG gene analysis may have biological and prognostic relevance. Hopefully, IG analysis will help tailor treatment strategies for the most aggressive cases.
hairy cell leukaemia, immunoglobulin genes, pathogenesis, prognosis
55-66
Forconi, Francesco
ce9ed873-58cf-4876-bf3a-9ba1d163edc8
June 2011
Forconi, Francesco
ce9ed873-58cf-4876-bf3a-9ba1d163edc8
Forconi, Francesco
(2011)
Hairy cell leukaemia: biological and clinical overview from immunogenetic insights.
Hematological Oncology, 29 (2), .
(doi:10.1002/hon.975).
(PMID:21674564)
Abstract
Hairy cell leukaemia (HCL) is a rare neoplasm of peripheral B cells which represents a paradox in oncology. Despite its largely unknown origin and behaviour, HCL is one of the few example of dramatic success in the treatment of a malignancy. The recent steps forward to understanding the biology of HCL from immunogenetic and genomic studies have recently provided new insight into diagnosis and prognosis. Several data from immunoglobulin gene (IG) analysis have provided hints regarding the cell of origin and the ongoing selective interactions of the tumour BCR with environmental stimuli. It has also 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 a central role of the tumour B-cell receptor in defining the outcome of HCL and that that IG gene analysis may have biological and prognostic relevance. Hopefully, IG analysis will help tailor treatment strategies for the most aggressive cases.
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e-pub ahead of print date: 2 November 2010
Published date: June 2011
Keywords:
hairy cell leukaemia, immunoglobulin genes, pathogenesis, prognosis
Organisations:
Cancer Sciences
Identifiers
Local EPrints ID: 358124
URI: http://eprints.soton.ac.uk/id/eprint/358124
ISSN: 0278-0232
PURE UUID: b46c0306-6156-43e4-9fa3-d130acdc0113
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Date deposited: 08 Oct 2013 09:59
Last modified: 15 Mar 2024 03:40
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