Syndrome de Richter

Cet article est une ebauche concernant la medecine .

Vous pouvez partager vos connaissances en l’ameliorant ( comment ? ) selon les recommandations des projets correspondants .

Le syndrome de Richter est la survenue d'un lymphome de haut grade au cours d'une leucemie lymphoide chronique . Il s'observe dans 3 a 10 % des cas. L'apparition d'un syndrome de Richter classe immediatement la LLC en stade C de la classification de Binet . Il a ete decrit en 1928 par le pathologiste americain M.N. Richter.

Physiopathologie [ modifier | modifier le code ]

La transformation d'une leucemie lymphoide chronique en syndrome de Richter serait favorisee par l'inactivation du TP53 et de CDKN2A ^[
1
]. La mutation du NOTCH1 pourrait egalement jouer un role ^[
2
].

Traitement [ modifier | modifier le code ]

Plusieurs combinaisons de chimiotherapie sont proposees : oxaliplatine - fludarabine - cytarabine - rituximab ^[
3
], cyclophosphamide - vincristine - doxorubicine - dexamethasone ^[
4
] ou rituximab - cyclophosphamide - vincristine - doxorubicine - prednisolone ^[
5
], toutes donnant une duree de remission courte.

La greffe de moelle doit etre proposee, quand cela est possible, des la remission atteinte ^[
6
].

Notes et references [ modifier | modifier le code ]

↑ 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
↑ 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
↑ 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
↑ 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
↑ 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
↑ 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

[Chigrinova_2013-1] 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

[Rossi_2012-2] 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

[Tsimberidou_2008-3] 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

[Dabaja_2001-4] 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

[Langerbeins_2014-5] 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

[Tsimberidou_2006-6] 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

[ 1 ]

[ 2 ]

[ 3 ]

[ 4 ]

[ 5 ]

[ 6 ]