Diagnostic and therapeutic challenges in hairy cell leukaemia-variant: where are we in 2021?

Expert Rev Hematol. 2021 Mar 24. doi: 10.1080/17474086.2021.1908121. Online ahead of print.ABSTRACTINTRODUCTION: Hairy cell leukemia-variant (HCL-V) is a rare B-cell neoplasm arising or homing primarily in the spleen. It has been considered in the WHO classification of Haemopoietic and Lymphoid Tumours as a provisional entity since 2008 and included under the umbrella of unclassifiable splenomegalic B-cell leukemia/lymphomas. The diagnosis is a challenge to haematopathologists and management of these patients by the clinicians is difficult due to the lack of diagnostic and therapeutic guidelines and prospective studies.AREAS COVERED: This manuscript is a comprehensive review of the clinical features, pathology, immunophenotypic profile, genomic alterations and therapeutic options of HCL-V. Diagnostic and therapeutic dilemmas are extensively outlined considering the information derived from a literature search covering from 1980 to 2019. Integration of all the data is needed and recommended for establishing the diagnosis of this leukemia.EXPERT OPINION: More extensive information of genomic aberrations underlying the pathogenesis of the disease would be a solid stone for the diagnosis. To this end, a collaborative work among scientists and pathologists from different centers is required and expected. In turn, this might have a relevant clinical translation by allowing to identify putative targets for therapy and to improve the outlook of these patients.PMID:33759673 | DOI:10.1...
Source: Expert Review of Hematology - Category: Hematology Authors: Source Type: research