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The genomics of hairy cell Leukaemia and splenic diffuse red pulp Lymphoma

The genomics of hairy cell Leukaemia and splenic diffuse red pulp Lymphoma
The genomics of hairy cell Leukaemia and splenic diffuse red pulp Lymphoma
Classical hairy cell leukaemia (HCLc), its variant form (HCLv), and splenic diffuse red pulp lymphoma (SDRPL) constitute a subset of relatively indolent B cell tumours, with low incidence rates of high-grade transformations, which primarily involve the spleen and bone marrow and are usually associated with circulating tumour cells characterised by villous or irregular cytoplasmic borders. The primary aim of this review is to summarise their cytogenetic, genomic, immunogenetic, and epigenetic features, with a particular focus on the clonal BRAFV600E mutation, present in most cases currently diagnosed with HCLc. We then reflect on their cell of origin and pathogenesis as well as present the clinical implications of improved biological understanding, extending from diagnosis to prognosis assessment and therapy response.
BRAFV600E, HCLc, HCLv, Hairy cell leukaemia, SDRPL, Tbet
2072-6694
Oscier, David
2e7f0cc1-93e2-441e-857d-7314efae08ec
Stamatopoulos, Kostas
04185c9f-28ac-4693-bcff-4b6f2081de5c
Mirandari, Amatta
b8640936-4b35-47c5-b6f1-f4d81ab6e2f6
Strefford, Jonathan
3782b392-f080-42bf-bdca-8aa5d6ca532f
Oscier, David
2e7f0cc1-93e2-441e-857d-7314efae08ec
Stamatopoulos, Kostas
04185c9f-28ac-4693-bcff-4b6f2081de5c
Mirandari, Amatta
b8640936-4b35-47c5-b6f1-f4d81ab6e2f6
Strefford, Jonathan
3782b392-f080-42bf-bdca-8aa5d6ca532f

Oscier, David, Stamatopoulos, Kostas, Mirandari, Amatta and Strefford, Jonathan (2022) The genomics of hairy cell Leukaemia and splenic diffuse red pulp Lymphoma. Cancers, 14 (3), [697]. (doi:10.3390/cancers14030697).

Record type: Review

Abstract

Classical hairy cell leukaemia (HCLc), its variant form (HCLv), and splenic diffuse red pulp lymphoma (SDRPL) constitute a subset of relatively indolent B cell tumours, with low incidence rates of high-grade transformations, which primarily involve the spleen and bone marrow and are usually associated with circulating tumour cells characterised by villous or irregular cytoplasmic borders. The primary aim of this review is to summarise their cytogenetic, genomic, immunogenetic, and epigenetic features, with a particular focus on the clonal BRAFV600E mutation, present in most cases currently diagnosed with HCLc. We then reflect on their cell of origin and pathogenesis as well as present the clinical implications of improved biological understanding, extending from diagnosis to prognosis assessment and therapy response.

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cancers-14-00697-v3 - Version of Record
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Accepted/In Press date: 25 January 2022
Published date: 29 January 2022
Keywords: BRAFV600E, HCLc, HCLv, Hairy cell leukaemia, SDRPL, Tbet

Identifiers

Local EPrints ID: 472540
URI: http://eprints.soton.ac.uk/id/eprint/472540
ISSN: 2072-6694
PURE UUID: 4a05297f-ab20-445c-928a-9d4469df5572
ORCID for Jonathan Strefford: ORCID iD orcid.org/0000-0002-0972-2881

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Date deposited: 07 Dec 2022 18:06
Last modified: 17 Mar 2024 02:59

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Contributors

Author: David Oscier
Author: Kostas Stamatopoulos
Author: Amatta Mirandari

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