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Hairy cell leukemia and COVID-19 adaptation of treatment guidelines.

ABSTRACT: Standard treatment options in classic HCL (cHCL) result in high response rates and near normal life expectancy. However, the disease itself and the recommended standard treatment are associated with profound and prolonged immunosuppression, increasing susceptibility to infections and the risk for a severe course of COVID-19. The Hairy Cell Leukemia Foundation (HCLF) has recently convened experts and discussed different clinical strategies for the management of these patients. The new recommendations adapt the 2017 consensus for the diagnosis and management with cHCL to the current COVID-19 pandemic. They underline the option of active surveillance in patients with low but stable blood counts, consider the use of targeted and non-immunosuppressive agents as first-line treatment for cHCL, and give recommendations on preventive measures against COVID-19.

SUBMITTER: Grever M

PROVIDER: S-EPMC8093591 | biostudies-literature | 2021 Jul

REPOSITORIES: biostudies-literature

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Hairy cell leukemia and COVID-19 adaptation of treatment guidelines.

Grever Michael M Andritsos Leslie L Banerji Versha V Barrientos Jacqueline C JC Bhat Seema S Blachly James S JS Call Timothy T Cross Matthew M Dearden Claire C Demeter Judit J Dietrich Sasha S Falini Brunangelo B Forconi Francesco F Gladstone Douglas E DE Gozzetti Alessandro A Iyengar Sunil S Johnston James B JB Juliusson Gunnar G Kraut Eric E Kreitman Robert J RJ Lauria Francesco F Lozanski Gerard G Parikh Sameer A SA Park Jae J Polliack Aaron A Ravandi Farhad F Robak Tadeusz T Rogers Kerry A KA Saven Alan A Seymour John F JF Tadmor Tamar T Tallman Martin S MS Tam Constantine S CS Tiacci Enrico E Troussard Xavier X Zent Clive C Zenz Thorsten T Zinzani Pier Luigi PL Wörmann Bernhard B

Leukemia 20210504 7

Standard treatment options in classic HCL (cHCL) result in high response rates and near normal life expectancy. However, the disease itself and the recommended standard treatment are associated with profound and prolonged immunosuppression, increasing susceptibility to infections and the risk for a severe course of COVID-19. The Hairy Cell Leukemia Foundation (HCLF) has recently convened experts and discussed different clinical strategies for the management of these patients. The new recommendat ...[more]

PMID: 33947938

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Project description:Hairy cell leukemia (HCL) is a chronic mature B-cell neoplasm with unique clinicopathologic features and an initial exquisite sensitivity to chemotherapy with purine analogs; however, the disease relapses, often repeatedly. The enigmatic pathogenesis of HCL was recently clarified by the discovery of its underlying genetic cause, the BRAF-V600E kinase-activating mutation, which is somatically and clonally present in almost all patients through the entire disease spectrum and clinical course. By aberrantly activating the RAF-MEK-ERK signaling pathway, BRAF-V600E shapes key biologic features of HCL, including its specific expression signature, hairy morphology, and antiapoptotic behavior. Accompanying mutations of the KLF2 transcription factor or the CDKN1B/p27 cell cycle inhibitor are recurrent in 16% of patients with HCL and likely cooperate with BRAF-V600E in HCL pathogenesis. Conversely, BRAF-V600E is absent in other B-cell neoplasms, including mimickers of HCL that require different treatments (eg, HCL-variant and splenic marginal zone lymphoma). Thus, testing for BRAF-V600E allows for a genetics-based differential diagnosis between HCL and HCL-like tumors, even noninvasively in routine blood samples. BRAF-V600E also represents a new therapeutic target. Patients' leukemic cells exposed ex vivo to BRAF inhibitors are spoiled of their HCL identity and then undergo apoptosis. In clinical trials of patients with HCL who have experienced multiple relapses after purine analogs or who are refractory to purine analogs, a short course of the oral BRAF inhibitor vemurafenib produced an almost 100% response rate, including complete remission rates of 35% to 42%, without myelotoxicity. To further improve on these results, it will be important to clarify the mechanisms of incomplete leukemic cell eradication by vemurafenib and to explore chemotherapy-free combinations of a BRAF inhibitor with other targeted agents (eg, a MEK inhibitor and/or an anti-CD20 monoclonal antibody).

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Hairy cell leukemia and COVID-19 adaptation of treatment guidelines.

Publications

Hairy cell leukemia and COVID-19 adaptation of treatment guidelines.

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