Syndrome de Richter

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Le syndrome de Richter est la survenue d'un lymphome de haut grade au cours d'une leucémie lymphoïde chronique. Il s'observe dans 3 à 10 % des cas. L'apparition d'un syndrome de Richter classe immédiatement la LLC en stade C de la classification de Binet. Il a été décrit en 1928 par le pathologiste américain M.N. Richter.

Physiopathologie

La transformation d'une leucémie lymphoïde chronique en syndrome de Richter serait favorisée par l'inactivation du TP53 et de CDKN2A[1]. La mutation du NOTCH1 pourrait également jouer un rôle[2].

Traitement

Plusieurs combinaisons de chimiothérapie sont proposées : oxaliplatine-fludarabine-cytarabine-rituximab[3], cyclophosphamide-vincristine-doxorubicine-dexaméthasone[4] ou rituximab-cyclophosphamide-vincristine-doxorubicine-prednisolone[5], toutes donnant une durée de rémission courte.

La greffe de moelle doit être proposée, quand cela est possible, dès la rémission atteinte[6].

Notes et références

  1. ↑ Chigrinova E, Rinaldi A, Kwee I et al. Two main genetic pathways lead to the transformation of chronic lymphocytic leukemia to Richter syndrome, Blood, 2013;122:2673–2682
  2. ↑ Rossi D, Rasi S, Spina V et al. Different impact of NOTCH1 and SF3B1 mutations on the risk of chronic lymphocytic leukemia transformation to Richter syndrome, Br J Haematol, 2012;158:426–429
  3. ↑ Tsimberidou AM, Wierda WG, Plunkett W et al. Phase I-II study of oxaliplatin, fludarabine, cytarabine, and rituximab combination therapy in patients with Richter's syndrome or fludarabine-refractory chronic lymphocytic leukemia; J Clin Oncol; 2008;26:196–203
  4. ↑ Dabaja BS, O'Brien SM, Kantarjian HM et al. Fractionated cyclophosphamide, vincristine, liposomal daunorubicin (daunoXome), and dexamethasone (hyperCVXD) regimen in Richter's syndrome, Leuk Lymphoma, 2001;42:329–337
  5. ↑ Langerbeins P, Busch R, Anheier N et al. Poor efficacy and tolerability of R-CHOP in relapsed/refractory chronic lymphocytic leukemia and Richter transformation, Am J Hematol, 2014;89:E239–E243
  6. ↑ Tsimberidou AM, O'Brien S, Khouri I et al. Clinical outcomes and prognostic factors in patients with Richter's syndrome treated with chemotherapy or chemoimmunotherapy with or without stem-cell transplantation, J Clin Oncol, 2006;24:2343–2351

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