Syndrome de Richter dans le paysage canadien : perspectives cliniques et tendances émergentes
Résumé
Au cours de la dernière décennie, le traitement de la leucémie lymphoïde chronique (LLC) et de son équivalent lymphomateux, le lymphome à petits lymphocytes (LPL), a considérablement évolué. Le recours accru aux agents ciblés, tels que les inhibiteurs de la tyrosine kinase de Bruton (iTKB) et les inhibiteurs de la protéine B-cell lymphoma-2 (iBCL2), au détriment de l’immunochimiothérapie (ICT), a permis d’améliorer considérablement les résultats des patients. Malgré ces progrès, certains patients connaissent encore une transformation de la maladie vers une histologie plus agressive connue sous le nom de syndrome (ou transformation) de Richter (SR) et les résultats cliniques avec cette histologie restent médiocres, avec une survie globale (SG) médiane généralement inférieure à un an. Par conséquent, le SR représente un besoin important non comblé pour les patients atteints de LLC/LPL. Cette revue présentera les progrès récents dans la compréhension et la prise en charge du SR dans le contexte canadien, en mettant l’accent sur la transformation en lymphome diffus à grandes cellules B (LDGCB).
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