Recommendations for the Management of Patients with Hairy-Cell Leukemia and Hairy-Cell Leukemia-like Disorders: A Work by French-Speaking Experts and French Innovative Leukemia Organization (FILO) Group Article Swipe
Jérôme Paillassa
,
Elsa Maître
,
N Boudjerra
,
Abdallah Madani
,
Raihane Benlakhal
,
Thomas Matthes
,
Eric Van Den Neste
,
Laura Cailly
,
Luca Inchiappa
,
Mohammed Amine Bekadja
,
Cécile Tomowiak
,
Xavier Troussard
·
YOU?
·
· 2024
· Open Access
·
· DOI: https://doi.org/10.3390/cancers16122185
YOU?
·
· 2024
· Open Access
·
· DOI: https://doi.org/10.3390/cancers16122185
Introduction: Hairy-cell leukemia (HCL) is a rare B-cell chronic lymphoproliferative disorder (B-CLPD), whose favorable prognosis has changed with the use of purine nucleoside analogs (PNAs), such as cladribine (CDA) or pentostatin (P). However, some patients eventually relapse and over time HCL becomes resistant to chemotherapy. Many discoveries have been made in the pathophysiology of HCL during the last decade, especially in genomics, with the identification of the BRAFV600E mutation and cellular biology, including the importance of signaling pathways as well as tumor microenvironment. All of these new developments led to targeted treatments, especially BRAF inhibitors (BRAFis), MEK inhibitors (MEKis), Bruton’s tyrosine kinase (BTK) inhibitors (BTKis) and recombinant anti-CD22 immunoconjugates. Results: The following major changes or additions were introduced in these updated guidelines: the clinical relevance of the changes in the classification of splenic B-cell lymphomas and leukemias; the increasingly important diagnostic role of BRAFV600E mutation; and the prognostic role of the immunoglobulin (IG) variable (V) heavy chain (H) (IGHV) mutational status and repertory. We also wish to insist on the specific involvement of bones, skin, brain and/or cerebrospinal fluid (CSF) of the disease at diagnosis or during the follow-up, the novel targeted drugs (BRAFi and MEKi) used for HCL treatment, and the increasing role of minimal residual disease (MRD) assessment. Conclusion: Here we present recommendations for the diagnosis of HCL, treatment in first line and in relapsed/refractory patients as well as for HCL-like disorders including HCL variant (HCL-V)/splenic B-cell lymphomas/leukemias with prominent nucleoli (SBLPN) and splenic diffuse red pulp lymphoma (SDRPL).
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- Type
- article
- Language
- en
- Landing Page
- https://doi.org/10.3390/cancers16122185
- https://www.mdpi.com/2072-6694/16/12/2185/pdf?version=1718016015
- OA Status
- gold
- Cited By
- 3
- References
- 170
- Related Works
- 10
- OpenAlex ID
- https://openalex.org/W4399493382
All OpenAlex metadata
Raw OpenAlex JSON
- OpenAlex ID
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https://openalex.org/W4399493382Canonical identifier for this work in OpenAlex
- DOI
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https://doi.org/10.3390/cancers16122185Digital Object Identifier
- Title
-
Recommendations for the Management of Patients with Hairy-Cell Leukemia and Hairy-Cell Leukemia-like Disorders: A Work by French-Speaking Experts and French Innovative Leukemia Organization (FILO) GroupWork title
- Type
-
articleOpenAlex work type
- Language
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enPrimary language
- Publication year
-
2024Year of publication
- Publication date
-
2024-06-10Full publication date if available
- Authors
-
Jérôme Paillassa, Elsa Maître, N Boudjerra, Abdallah Madani, Raihane Benlakhal, Thomas Matthes, Eric Van Den Neste, Laura Cailly, Luca Inchiappa, Mohammed Amine Bekadja, Cécile Tomowiak, Xavier TroussardList of authors in order
- Landing page
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https://doi.org/10.3390/cancers16122185Publisher landing page
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https://www.mdpi.com/2072-6694/16/12/2185/pdf?version=1718016015Direct link to full text PDF
- Open access
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YesWhether a free full text is available
- OA status
-
goldOpen access status per OpenAlex
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https://www.mdpi.com/2072-6694/16/12/2185/pdf?version=1718016015Direct OA link when available
- Concepts
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Hairy cell leukemia, Leukemia, Work (physics), Medicine, Engineering, Immunology, Mechanical engineeringTop concepts (fields/topics) attached by OpenAlex
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3Total citation count in OpenAlex
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2025: 2, 2024: 1Per-year citation counts (last 5 years)
- References (count)
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170Number of works referenced by this work
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10Other works algorithmically related by OpenAlex
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| abstract_inverted_index.identification | 64 |
| abstract_inverted_index.immunoglobulin | 151 |
| abstract_inverted_index.(HCL-V)/splenic | 237 |
| abstract_inverted_index.pathophysiology | 52 |
| abstract_inverted_index.recommendations | 214 |
| abstract_inverted_index.immunoconjugates. | 108 |
| abstract_inverted_index.microenvironment. | 82 |
| abstract_inverted_index.lymphomas/leukemias | 239 |
| abstract_inverted_index.lymphoproliferative | 9 |
| abstract_inverted_index.relapsed/refractory | 226 |
| cited_by_percentile_year.max | 97 |
| cited_by_percentile_year.min | 90 |
| corresponding_author_ids | https://openalex.org/A5081871516 |
| countries_distinct_count | 5 |
| institutions_distinct_count | 12 |
| corresponding_institution_ids | https://openalex.org/I4210114068, https://openalex.org/I98702875 |
| sustainable_development_goals[0].id | https://metadata.un.org/sdg/3 |
| sustainable_development_goals[0].score | 0.49000000953674316 |
| sustainable_development_goals[0].display_name | Good health and well-being |
| citation_normalized_percentile.value | 0.85171554 |
| citation_normalized_percentile.is_in_top_1_percent | False |
| citation_normalized_percentile.is_in_top_10_percent | True |