Hairy cell leukemia 2024: Update on diagnosis, risk‐stratification, and treatment—Annual updates in hematological malignancies

Xavier Troussard; Elsa Maitre; Jérôme Paillassa

doi:10.1002/ajh.27240

journal article Mar 05, 2024

Hairy cell leukemia 2024: Update on diagnosis, risk‐stratification, and treatment—Annual updates in hematological malignancies

Xavier Troussard Elsa Maitre

Jérôme Paillassa

American Journal of Hematology Vol. 99 No. 4 pp. 679-696 · Wiley

View at Publisher Save 10.1002/ajh.27240

Abstract

AbstractDisease OverviewHairy cell leukemia (HCL) and HCL‐like disorders, including HCL variant (HCL‐V) and splenic diffuse red pulp lymphoma (SDRPL), are a very heterogenous 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.DiagnosisDiagnosis 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 bone marrow infiltration and the presence of BRAFV600E somatic mutation.Risk StratificationProgression 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, as well as HCL with TP53 mutations and HCL‐V.TreatmentPatients 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 cladribine (CDA) and rituximab (R) represents an increasingly used therapeutic approach. Management of relapsed/refractory disease is based on the use of BRAF inhibitors (BRAFi) plus R, MEK inhibitors (MEKi), recombinant immunoconjugates targeting CD22, Bruton tyrosine kinase inhibitors (BTKi), and Bcl‐2 inhibitors (Bcl‐2i). However, the optimal sequence of the different treatments remains to be determined.

Topics

No keywords indexed for this article. Browse by subject →

References

205

[1]

The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms

Rita Alaggio, Catalina Amador, Ioannis Anagnostopoulos et al.

Leukemia 10.1038/s41375-022-01620-2

[2]

10.1182/blood.2022015851

[3]

10.1111/j.1365-2141.1974.tb06769.x

[4]

10.3322/caac.21357

[5]

Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet—a population-based study

Gemma Gatta, Riccardo Capocaccia, Laura Botta et al.

The Lancet Oncology 10.1016/s1470-2045(17)30445-x

[6]

10.1182/blood-2006-11-051672

[7]

10.1111/j.1600-0609.2006.00732.x

[8]

10.1016/j.clml.2015.03.001

[9]

10.1182/blood-2017-12-820381

[10]

10.1016/j.ctarc.2020.100236

[11]

10.1002/ajh.26881

[12]

10.1016/j.clml.2017.07.003

[13]

10.1080/10428190290021560

[14]

10.1093/jncimonographs/lgu004

[15]

10.3109/10428194.2011.610011

[16]

10.5271/sjweh.143

[17]

10.1111/j.1365-2141.1995.tb05261.x

[18]

10.1016/j.beha.2015.10.014

[19]

10.1016/j.ctrv.2010.05.003

[20]

10.1080/17474086.2021.1908121

[21]

10.1182/blood-2007-07-098848

[22]

10.1182/blood-2018-06-857938

[23]

10.1182/bloodadvances.2023011147

[24]

10.1007/s00277-020-04349-z

[25]

10.5045/br.2020.2020069

[26]

10.1111/ejh.13505

[27]

10.1002/ccr3.7343

[28]

10.1515/cclm-2017-0763

[29]

Matutes E "The immunophenotype of hairy cell leukemia (HCL). Proposal for a scoring system to distinguish HCL from B‐cell disorders with hairy or villous lymphocytes" Leuk Lymphoma (1994)

[30]

10.1002/cyto.b.21365

[31]

10.1016/j.clml.2016.02.041

[32]

10.3390/cancers14041050

[33]

10.1159/000512830

[34]

10.1111/bjh.18224

[35]

10.1182/blood-2016-01-689422

[36]

10.1016/s0140-6736(04)16356-3

[37]

10.1111/bjh.15502

[38]

10.1182/blood.2021015066

[39]

10.1016/j.leukres.2012.11.021

[40]

10.1056/nejmoa1014209

[41]

10.3390/ijms22157780

[42]

10.1126/scitranslmed.3008004

[43]

10.1111/bjh.12735

[44]

10.1111/jcmm.17890

[45]

Mechanism of Activation of the RAF-ERK Signaling Pathway by Oncogenic Mutations of B-RAF

Paul T.C Wan, Mathew J Garnett, S.Mark Roe et al.

Cell 10.1016/s0092-8674(04)00215-6

[46]

10.1038/ng.2828

[47]

10.1182/blood-2015-04-643361

[48]

10.1182/blood-2017-01-765107

[49]

10.1182/blood-2011-08-371179

[50]

Piris MA (2008)

Showing 50 of 205 references

Metrics

19

Citations

205

References

Details

Published: Mar 05, 2024
Vol/Issue: 99(4)
Pages: 679-696
License: View

Authors

X

Xavier Troussard

Laboratoire Hématologie CHU Côte de Nacre Caen Cedex France

E

Elsa Maitre

Laboratoire Hématologie CHU Côte de Nacre Caen Cedex France

J

Jérôme Paillassa

Service des Maladies du Sang CHU Angers Angers Cedex France

Cite This Article

Xavier Troussard, Elsa Maitre, Jérôme Paillassa (2024). Hairy cell leukemia 2024: Update on diagnosis, risk‐stratification, and treatment—Annual updates in hematological malignancies. American Journal of Hematology, 99(4), 679-696. https://doi.org/10.1002/ajh.27240

Hairy cell leukemia 2024: Update on diagnosis, risk‐stratification, and treatment—Annual updates in hematological malignancies

You May Also Like