Differences among young adults, adults and elderly chronic myeloid leukemia patients.

Fausto Castagnetti,Gabriele Gugliotta,Michele Baccarani,Massimo Breccia,Giorgina Specchia,L Levato,Elisabetta Abruzzese, Gian Luigi Rossi,Alessandra Iurlo,Beniamino Di Martino,Patrizia Pregno,Fabio Stagno,A Cuneo,M D Bonifacio,Marco Gobbi,Domenico Russo,Antonella Gozzini,Mario Tiribelli,A De Vivo,Giuliana Alimena,Michele Cavo,G Martinelli,Fabrizio Pane,Giuseppe Saglio,Gianantonio Rosti,Flavia Salvi,M Pini, P Leoni,Serena Rupoli,Piero Galieni,Catia Bigazzi,Nicola Cantore,Francesco A N Palmieri,Francesco Albano, A Russo Rossi,Alessandro Rambaldi,Tamara Intermesoli,F Palandri,Nicoletta Testoni,Simona Luatti,Simona Soverini,Ilaria Iacobucci,Mt Bochicchio, M Apolinari,Miriam Fogli, I Cervello, A Capucci,Michele Malagola, A Malpignano, M Girasoli,Emanuele Angelucci,Emilio Usala, S Storti,E De Biasi,Giuseppe Tagariello,R Sartori,F Di Raimondo,Paolo Vigneri, S Impera,Stefano Molica,Francesco Lanza, Clara Vigano,Mariella Grasso,D Rapezzi,Francesco Cavazzini, A Bosi,Valeria Santini, Sf Capalbo, G Spinosa,Ivana Pierri,M Bergamaschi,Am Carella,Andrea Bacigalupo, A De Blasio, Flavia Helena Ciccone,N Di Renzo,Caterina Musolino, Suzana Leitao Russo,Agostino Cortelezzi,Enrica Morra, Em Pungolino,Mario Luppi,Roberto Marasca,Em Pogliani,Carlo Gambacortipasserini,Luigiana Luciano,Felicetto Ferrara, M Annunziata, G Latte, D Noli,Giovanna Regecambrin,Carmen Fava,Gianpietro Semenzato,Gianni Binotto,Francesco Fabbiano, D Turri,Sergio Siragusa, Claudia Caracciolo, M Musso,Ferdinando Porretto, F Aversa,M Crugnola,Mario Cazzola,Ester Orlandi,Brunangelo Falini,Franca Falzetti,Giuseppe Visani,Alessandro Isidori,Giuseppe Fioritoni,R Di Lorenzo,D Vallisa,Elena Trabacchi,Mario Petrini,Sara Galimberti,M Pizzuti,Alfonso Zaccaria,M Salvucci, F Ronco, D Ielo,Francesco Merli,P Avanzini,Patrizia Tosi, A Merli,Pellegrino Musto,V De Stefano,Simona Sica,Roberto Latagliata,P De Fabritiis, M Trawiska,Ignazio Majolino, L Pacilli, B Ronci,Michele Cedrone, Mc Petti,Francesco Pisani,Agostino Tafuri,Enrico Montefusco, F Iuliano, F Dore,Simonetta Pardini,Monica Bocchia,Marzia Defina,Am Liberati,D Luzzi,Mario Boccadoro,Dario Ferrero,Umberto Vitolo,F Gherlinzoni,Elisabetta Calistri,Renato Fanin,Giovanni Pizzolo, Vittorio Meneghini, F Rodighiero,A Demilio

Annals of Oncology(2015)

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摘要
The incidence of chronic myeloid leukemia (CML) increases with age, but it is unclear how the characteristics of the disease vary with age. In children, where CML is very rare, it presents with more aggressive features, including huge splenomegaly, higher cell count and higher blast cell percentage. To investigate if after childhood the disease maintains or loses these characteristics of aggressiveness, we analyzed 2784 adult patients, at least 18 years old, registered by GIMEMA CML WP over a 40-year period. Young adults (YAs: 18-29 years old) significantly differed from adults (30-59 years old) and elderly patients (at least 60 years old) particularly for the frequency of splenomegaly (71%, 63% and 55%, P < 0.001), and the greater spleen size (median value: 4.5, 3.0 and 1.0 cm, P < 0.001). According to the EUTOS score, that is age-independent, high-risk patients were more frequent among YAs, than among adult and elderly patients (18%, 9% and 6%, P < 0.001). In tyrosine kinase inhibitors-treated patients, the rates of complete cytogenetic and major molecular response were lower in YAs, and the probability of transformation was higher (16%, 5% and 7%, P = 0.011). The characteristics of CML or the host response to leukemia differ with age. The knowledge of these differences and of their causes may help to refine the treatment and to improve the outcome.
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关键词
chronic myeloid leukemia,BCR-ABL,prognosis,young adults,tyrosine kinase inhibitors
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