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Insight into the behavior of hairy cell leukemia by immunogenetic analysis

Insight into the behavior of hairy cell leukemia by immunogenetic analysis
Insight into the behavior of hairy cell leukemia by immunogenetic analysis
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 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 (i) have variable levels of IG somatic mutations, (ii) continue somatic mutations at low levels, (iii) have active processes of IG class switching after transformation, and (iv) have a relatively high frequency of selective events in the light chain of the BCR. It has recently emerged that an unmutated status of the HCL-derived IG can be associated with failure to respond to cladribine, genetic abnormalities indicative of poor outcome, and aggressive disease. These observations suggest that IG gene analysis may have biological and prognostic relevance in HCL and merits further characterization.
hairy cell leukemia, cladribine, igh, immunogenetics, prognosis
1042-8194
103-107
Forconi, Francesco
ce9ed873-58cf-4876-bf3a-9ba1d163edc8
Cencini, Emanuele
3dc5d188-96b0-4f7d-abb8-af4d22638d7e
Sozzi, Elisa
e811ff23-6fe9-47ec-b052-fe8d34d1e36b
Sicuranza, Anna
4ffad2b1-244f-4813-ac84-f573f8f44409
Raspadori, Donatella
185f6866-6d10-4f86-9add-72938cea80b3
Lauria, Francesco
f01f163b-abcb-4aad-b952-f4356692f519
Forconi, Francesco
ce9ed873-58cf-4876-bf3a-9ba1d163edc8
Cencini, Emanuele
3dc5d188-96b0-4f7d-abb8-af4d22638d7e
Sozzi, Elisa
e811ff23-6fe9-47ec-b052-fe8d34d1e36b
Sicuranza, Anna
4ffad2b1-244f-4813-ac84-f573f8f44409
Raspadori, Donatella
185f6866-6d10-4f86-9add-72938cea80b3
Lauria, Francesco
f01f163b-abcb-4aad-b952-f4356692f519

Forconi, Francesco, Cencini, Emanuele, Sozzi, Elisa, Sicuranza, Anna, Raspadori, Donatella and Lauria, Francesco (2011) Insight into the behavior of hairy cell leukemia by immunogenetic analysis. Leukemia and Lymphoma, 52, supplement 2, 103-107. (doi:10.3109/10428194.2011.569620). (PMID:21504291)

Record type: Article

Abstract

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 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 (i) have variable levels of IG somatic mutations, (ii) continue somatic mutations at low levels, (iii) have active processes of IG class switching after transformation, and (iv) have a relatively high frequency of selective events in the light chain of the BCR. It has recently emerged that an unmutated status of the HCL-derived IG can be associated with failure to respond to cladribine, genetic abnormalities indicative of poor outcome, and aggressive disease. These observations suggest that IG gene analysis may have biological and prognostic relevance in HCL and merits further characterization.

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More information

Published date: June 2011
Keywords: hairy cell leukemia, cladribine, igh, immunogenetics, prognosis
Organisations: Cancer Sciences

Identifiers

Local EPrints ID: 358126
URI: http://eprints.soton.ac.uk/id/eprint/358126
ISSN: 1042-8194
PURE UUID: b19e7a7a-2406-44be-8978-73619950c4c6
ORCID for Francesco Forconi: ORCID iD orcid.org/0000-0002-2211-1831

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Date deposited: 08 Oct 2013 10:02
Last modified: 15 Mar 2024 03:40

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Contributors

Author: Emanuele Cencini
Author: Elisa Sozzi
Author: Anna Sicuranza
Author: Donatella Raspadori
Author: Francesco Lauria

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