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Review

The Biology of Classic Hairy Cell Leukemia

1
Department of Internal Medicine V, Hematology and Oncology, Medical University of Innsbruck, 6020 Innsbruck, Austria
2
Experimental Oncogenomic Group, Tyrolean Cancer Research Institute, 6020 Innsbruck, Austria
3
Department of Hematology and Oncology, Medical Clinic 3, University Hospital Bonn, 53127 Bonn, Germany
*
Author to whom correspondence should be addressed.
Academic Editor: Athanasia Mouzaki
Int. J. Mol. Sci. 2021, 22(15), 7780; https://doi.org/10.3390/ijms22157780
Received: 22 June 2021 / Revised: 12 July 2021 / Accepted: 19 July 2021 / Published: 21 July 2021
(This article belongs to the Special Issue Immunological Investigations in Hematology)
Classic hairy cell leukemia (HCL) is a rare mature B-cell malignancy associated with pancytopenia and infectious complications due to progressive infiltration of the bone marrow and spleen. Despite tremendous therapeutic advances achieved with the implementation of purine analogues such as cladribine into clinical practice, the culprit biologic alterations driving this fascinating hematologic disease have long stayed concealed. Nearly 10 years ago, BRAF V600E was finally identified as a key activating mutation detectable in almost all HCL patients and throughout the entire course of the disease. However, additional oncogenic biologic features seem mandatory to enable HCL transformation, an open issue still under active investigation. This review summarizes the current understanding of key pathogenic mechanisms implicated in HCL and discusses major hurdles to overcome in the context of other BRAF-mutated malignancies. View Full-Text
Keywords: hairy cell leukemia; HCL; biology; microenvironment; BRAF V600E; DUSP; single-cell sequencing; vitronectin; fibronectin; JNK; p38; B-cell receptor; epigenetic; methylome; microRNA hairy cell leukemia; HCL; biology; microenvironment; BRAF V600E; DUSP; single-cell sequencing; vitronectin; fibronectin; JNK; p38; B-cell receptor; epigenetic; methylome; microRNA
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MDPI and ACS Style

Bohn, J.-P.; Salcher, S.; Pircher, A.; Untergasser, G.; Wolf, D. The Biology of Classic Hairy Cell Leukemia. Int. J. Mol. Sci. 2021, 22, 7780. https://doi.org/10.3390/ijms22157780

AMA Style

Bohn J-P, Salcher S, Pircher A, Untergasser G, Wolf D. The Biology of Classic Hairy Cell Leukemia. International Journal of Molecular Sciences. 2021; 22(15):7780. https://doi.org/10.3390/ijms22157780

Chicago/Turabian Style

Bohn, Jan-Paul, Stefan Salcher, Andreas Pircher, Gerold Untergasser, and Dominik Wolf. 2021. "The Biology of Classic Hairy Cell Leukemia" International Journal of Molecular Sciences 22, no. 15: 7780. https://doi.org/10.3390/ijms22157780

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