Leucémie à tricholeucocytes et sa forme variante : prise en charge en 2024 (notice n° 1134411)
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|---|---|
| fixed length control field | 05108cam a2200433 4500500 |
| 005 - DATE AND TIME OF LATEST TRANSACTION | |
| control field | 20250413035221.0 |
| 041 ## - LANGUAGE CODE | |
| Language code of text/sound track or separate title | fre |
| 042 ## - AUTHENTICATION CODE | |
| Authentication code | dc |
| 100 10 - MAIN ENTRY--PERSONAL NAME | |
| Personal name | Inchiappa, Luca |
| Relator term | author |
| 245 00 - TITLE STATEMENT | |
| Title | Leucémie à tricholeucocytes et sa forme variante : prise en charge en 2024 |
| 260 ## - PUBLICATION, DISTRIBUTION, ETC. | |
| Date of publication, distribution, etc. | 2025.<br/> |
| 500 ## - GENERAL NOTE | |
| General note | 4 |
| 520 ## - SUMMARY, ETC. | |
| Summary, etc. | Hairy cell leukemia (HCL) is a rare form of leukemia that belongs to the group of mature and chronic B-cell lymphoid disorders. There are two distinct entities: the classic form and the variant form, each with different cytological, phenotypic, and molecular characteristics. Splenic diffuse red pulp lymphoma, a hairy cell proliferation, is very close to the variant form. The classic form is easily diagnosed with the currently available tools, as the BRAFV600E mutation is detected in more than 95% of patients. The introduction of purine analogs has revolutionized the prognosis of this disease, with overall survival curves approaching those of the general population. The current treatment of choice is cladribine in combination with rituximab, which has demonstrated the best outcomes in terms of complete remission (CR), progression-free survival (PFS) and minimal residual disease (MRD) negativity rates. However, relapses still occur and recent discoveries of targeted therapies have transformed the management of these HCL relapsed/refractory patients, in particular BRAF inhibitors (BRAFi) combined with anti-CD20 monoclonal antibodies. Other agents are available for subsequent relapses or for patients without the BRAFV600E mutation. The variant form has a less favorable prognosis, largely due to a poorer response to purine analogs. In cases of relapse, alternative therapies are available, although options are more limited. Finally, recent advances in the understanding of the pathogenic mechanisms of both forms including in signaling pathways are opening new therapeutic avenues? In this article, we present a review on the clinical and biological aspects of hairy cell leukemia and its variant form and we propose a treatment algorithm. |
| 520 ## - SUMMARY, ETC. | |
| Summary, etc. | La leucémie à tricholeucocytes (LT) est une forme rare de leucémie, faisant partie des hémopathies lymphoïdes chroniques de phénotype B mature. On distingue deux entités principales : la forme classique et la forme variante (LT-V). Ces deux formes présentent des caractéristiques cytologiques, phénotypiques et moléculaires distinctes. Le lymphome diffus de la pulpe rouge de la rate est une entité entrant dans le cadre des proliférations à cellules chevelues, proche de la LT-V. La forme classique de la LT est facilement reconnue, la mutation BRAFV600E étant présente chez plus de 95 % des patients. L’introduction des analogues de purines a changé son pronostic, l’espérance de vie des patients se rapprochant de celle de la population générale. Le traitement standard de première ligne est l’association de cladribine et de rituximab, qui offre les meilleurs résultats en termes de rémission complète, de survie sans progression et de négativation de la maladie résiduelle. Les rechutes restent cependant fréquentes et les traitements ciblés ont transformé la prise en charge de ces patients en rechute/réfractaires, notamment avec l’introduction des inhibiteurs de BRAF en association avec les anticorps monoclonaux anti-CD20. D’autres molécules sont aussi disponibles en cas de nouvelles rechutes ou chez les patients ne présentant pas la mutation BRAFV600E. La LT-V présente un pronostic moins favorable, en partie en raison d’une réponse moindre aux analogues des purines. En cas de rechute, des thérapies alternatives sont disponibles, même si les choix sont plus limités. Enfin, les récentes avancées dans la compréhension des mécanismes de la pathogenèse des deux formes, ainsi que des voies de signalisation impliquées, ouvrent de nouvelles perspectives thérapeutiques prometteuses. Nous présentons ici une revue sur les aspects cliniques et biologiques de la LT et de la LT-V, et nous proposons un algorithme de traitement. |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | (MAP2K1) |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | 600E |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | analogues des purines |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | BRAFV |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | forme variante de la leucémie à<br/>tricholeucocytes (LT-V) |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | inhibiteurs de BRAF |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | Leucémie à tricholeucocytes (LT) |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | mitogen-activated protein kinase kinase 1 |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | BRAF |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | BRAF inhibitors |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | Hairy cell leukemia (HCL) |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | hairy cell leukemia variant |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | MAP2K1 mutations |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | mutation |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | purine analogs |
| 690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
| Topical term or geographic name as entry element | V600E |
| 700 10 - ADDED ENTRY--PERSONAL NAME | |
| Personal name | Paillassa, Jérôme |
| Relator term | author |
| 700 10 - ADDED ENTRY--PERSONAL NAME | |
| Personal name | Maitre, Elsa |
| Relator term | author |
| 700 10 - ADDED ENTRY--PERSONAL NAME | |
| Personal name | Tauveron-Jalenques, Urbain |
| Relator term | author |
| 700 10 - ADDED ENTRY--PERSONAL NAME | |
| Personal name | Tomowiak, Cécile |
| Relator term | author |
| 700 10 - ADDED ENTRY--PERSONAL NAME | |
| Personal name | Kernevès, Pauline |
| Relator term | author |
| 700 10 - ADDED ENTRY--PERSONAL NAME | |
| Personal name | Troussard, Xavier |
| Relator term | author |
| 786 0# - DATA SOURCE ENTRY | |
| Note | Hématologie | 31 | 1 | 2025-03-31 | p. 43-59 | 1264-7527 |
| 856 41 - ELECTRONIC LOCATION AND ACCESS | |
| Uniform Resource Identifier | <a href="https://shs.cairn.info/revue-hematologie-2025-1-page-43?lang=fr&redirect-ssocas=7080">https://shs.cairn.info/revue-hematologie-2025-1-page-43?lang=fr&redirect-ssocas=7080</a> |
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