Variant form of hairy cell leukemia

Wiber, Margaux; Maître, Elsa; Cornet, Édouard; Salaün, Véronique; Naguib, Dina; Troussard, Xavier

doi:10.1002/ccr3.2176

Cited by 9 publications

(8 citation statements)

References 21 publications

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“…The same scheme could be followed in case of relapse. Ibrutinib could represent an alternative therapy either at first‐line and relapse 98,99 . A clinical case report also demonstrated the interest of venetoclax in a biclonal IGHV4‐34 HCL‐V associated with CLL 100 …”

Section: Update On Hcl Treatment and Hcl‐like Disordersmentioning

confidence: 92%

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Hairy cell leukemia 2022: Update on diagnosis, risk‐stratification, and treatment

2021

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Disease Overview: Hairy cell leukemia (HCL) and HCL-like disorders, including HCL variant (HCL-V) and splenic diffuse red pulp lymphoma (SDRPL), are a very heterogeneous group of mature lymphoid B-cell disorders characterized by the identification of hairy cells, a specific genetic profile, a different clinical course, and the need for appropriate treatment. Diagnosis: Diagnosis of HCL is based on morphological evidence of hairy cells, an HCL immunologic score of 3 or 4 based on the CD11C, CD103, CD123, and CD25 expression, the trephine biopsy which makes it possible to specify the degree of tumoral medullary infiltration and the presence of BRAF V600E somatic mutation. Risk Stratification: Progression of patients with HCL is based on a large splenomegaly, leukocytosis, a high number of hairy cells in the peripheral blood, and the immunoglobulin heavy chain variable region gene mutational status. VH4-34-positive HCL cases are associated with a poor prognosis. Treatment: Patients should be treated only if HCL is symptomatic. Chemotherapy with risk adapted therapy purine analogs (PNAs) are indicated in first-line HCL patients. The use of chemo-immunotherapy combining PNAs and rituximab (R) represents an increasingly used therapeutic approach. Management of relapsed/ refractory disease is based on the use of BRAF inhibitors (BRAFi) plus rituximab or MEK inhibitors (MEKi), recombinant immunoconjugates targeting CD22 or BrutonTyrosine Kinase inhibitors (BTKi). However, the optimal sequence of the different treatments remains to be determined. The Bcl2-inhibitors (Bcl-2i) can play a major role in the future. | INTRODUCTIONHairy cell leukemia (HCL) is recognized as an entity by the World Health Organization since 2008 1 and in the last 2017 revision of the WHO classification of lymphoid neoplasms. 2 HCL, four to five times more frequent in men than women, accounts for 2% of all leukemias with approximately 1.100 new HCL cases in the United States. 3 The few available population-based studies are limited. [4][5][6][7][8] When including HCL and HCL-like disorders, the overall age-adjusted to the 2000 US population incidence rate is 0.7 per 100 000 in men and 0.3/100 000 in women. When adjusting to the worldwide population, the incidence rate of HCL is 0.3 and 0.1/100 000, respectively, and remains relatively stable over time. 8 It is lower in non-Hispanic/black, Hispanic, or Asian/pacific Islander. Despite an improvement of overall (OS) and relative survival (RS), a significantly lower OS is observed among African American individuals compared with other ethnic groups. 6 The mortality rates for patients with HCL is similar to those of the general population 5 years after diagnosis. 9 HCL must be differentiated from other

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Section: Update On Hcl Treatment and Hcl‐like Disordersmentioning

confidence: 92%

“…Ibrutinib could represent an alternative therapy either at first-line and relapse. 98,99 A clinical case report also demonstrated the interest of venetoclax in a biclonal IGHV4-34…”

Section: Hcl-v and Sdrplmentioning

confidence: 98%

Hairy cell leukemia 2022: Update on diagnosis, risk‐stratification, and treatment

2021

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“…55 Individual case reports of ibrutinib in patients with HCL-V show promising effects and warrant further investigation. 56,57…”

Section: Ibrutinibmentioning

confidence: 99%

“…In a phase II trial of 17 cases of relapsed/refractory HCL, ORR was 60% (25% CR and 35% PR) after prolonged administration of the drug 55 . Individual case reports of ibrutinib in patients with HCL‐V show promising effects and warrant further investigation 56,57 …”

Section: Experimental Approaches and Clinical Trialsmentioning

confidence: 99%

Guideline for diagnosis and management of hairy cell leukaemia (HCL) and hairy cell variant (HCL‐V)

et al. 2020

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This guideline was compiled according to the BSH process (https://b-s-h.org.uk/guidelines). The Grading of Recommendations Assessment, Development and Evaluation (GRADE) nomenclature was used to evaluate levels of evidence and to assess the strength of recommendations. The GRADE criteria can be found at http://www.gradeworkinggroup.org. Literature review A literature search was undertaken by Niche Science and Technology Ltd on June 30, 2018, using PubMed, with a further check by the authors on March 1, 2020. The following search terms were used: hairy cell leukaemia AND; BRAF V600E mutation, vemurafenib, dabrafenib, immunotherapy, rituximab, immunotoxin conjugate, moxetumomab, minimal residual disease, hairy cell leukaemia variant. Review of the manuscript Review of the manuscript was performed by the British Society for Haematology (BSH) Guidelines Committee Haemato-Oncology Taskforce, the BSH Guidelines Committee and the Haemato-Oncology Sounding Board of BSH. It was also placed on the members section of the BSH website for comment, and has been reviewed by the Leukaemia Care patient advocacy group. Diagnostic tests Blood film, classical HCL The peripheral blood film may show characteristic mediumsized lymphoid cells with an oval or indented (kidney-shaped)

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“… 2 , 12 Patients tend to develop cytopenias as a result of hypersplenism rather than bone marrow failure. 9 , 13 As noted above, while there may be a number of commonalities between the initial presentations of HCL-c and HCL-v, the clinical course of HCL-v is more aggressive, 10 with a medial overall survival (OS) of 9 years 11 , 14 in contrast to HCL-c in which patients may not experience a decline in OS due to improvements in therapies. 15 , 16 …”

Section: Introductionmentioning

confidence: 99%

Current and Emerging Therapeutic Options for Hairy Cell Leukemia Variant

Liu

Harris

Epperla

et al. 2021

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Hairy cell leukemia variant (HCL-v) is a rare B-cell lymphoproliferative disorder with distinct immunophenotypic and molecular characteristics when compared to classical hairy cell leukemia (HCL-c). In contrast to the enormous progress in therapeutic options for HCL-c, HCL-v remains a therapeutic challenge due to inferior outcomes with standard chemoimmunotherapy and BCR signaling pathway inhibitors, and due to the fact that HCL-v has limited molecular therapeutic targets. In addition, because of the rarity of the disease, there is a paucity of later phase studies or multicenter trials to guide treatment decisions. In this article, we briefly review the diagnostic criteria and clinical characteristics of HCL-v and present a comprehensive overview of current therapeutic options in HCL-v.

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Variant form of hairy cell leukemia

Cited by 9 publications

References 21 publications

Hairy cell leukemia 2022: Update on diagnosis, risk‐stratification, and treatment

Hairy cell leukemia 2022: Update on diagnosis, risk‐stratification, and treatment

Guideline for diagnosis and management of hairy cell leukaemia (HCL) and hairy cell variant (HCL‐V)

Current and Emerging Therapeutic Options for Hairy Cell Leukemia Variant

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