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Schanz J.,University of Gottingen | Tuchler H.,L Boltzmann Institute For Leukemia Research | Sole F.,Hospital del Mar | Mallo M.,Hospital del Mar | And 24 more authors.
Journal of Clinical Oncology | Year: 2012

Purpose: The karyotype is a strong independent prognostic factor in myelodysplastic syndromes (MDS). Since the implementation of the International Prognostic Scoring System (IPSS) in 1997, knowledge concerning the prognostic impact of abnormalities has increased substantially. The present study proposes a new and comprehensive cytogenetic scoring system based on an international data collection of 2,902 patients. Patients and Methods: Patients were included from the German-Austrian MDS Study Group (n = 1,193), the International MDS Risk Analysis Workshop (n = 816), the Spanish Hematological Cytogenetics Working Group (n = 849), and the International Working Group on MDS Cytogenetics (n = 44) databases. Patients with primary MDS and oligoblastic acute myeloid leukemia (AML) after MDS treated with supportive care only were evaluated for overall survival (OS) and AML evolution. Internal validation by bootstrap analysis and external validation in an independent patient cohort were performed to confirm the results. Results: In total, 19 cytogenetic categories were defined, providing clear prognostic classification in 91% of all patients. The abnormalities were classified into five prognostic subgroups (P < .001): very good (median OS, 61 months; hazard ratio [HR], 0.5; n = 81); good (49 months; HR, 1.0 [reference category]; n = 1,809); intermediate (26 months; HR, 1.6; n = 529); poor (16 months; HR, 2.6; n = 148); and very poor (6 months; HR, 4.2; n = 187). The internal and external validations confirmed the results of the score. Conclusion: In conclusion, these data should contribute to the ongoing efforts to update the IPSS by refining the cytogenetic risk categories. © 2012 by American Society of Clinical Oncology.


Ribera J.-M.,Autonomous University of Barcelona | Ribera J.-M.,Jose Carreras Leukemia Research Institute | Ribera J.,Jose Carreras Leukemia Research Institute | Genesca E.,Jose Carreras Leukemia Research Institute
Mediterranean Journal of Hematology and Infectious Diseases | Year: 2014

The primary objective of this review was to update and discuss the current concepts and the results of the treatment of acute lymphoblastic leukemia (ALL) in adolescents and young adults (AYA). After a brief consideration of the epidemiologic and clinicobiologic characteristics of ALL in the AYA population, the main retrospective comparative studies stating the superiority of pediatric over adult-based protocols were reviewed. The most important prospective studies in young adults using pediatric inspired or pediatric unmodified protocols were also reviewed emphasizing their feasibility at least up to the age of 40 yr and their promising results, with event-free survival rates of 60-65% or greater. Results of trials from pediatric groups have shown that the unfavourable prognosis of adolescents is no more adequate. The majority of the older adolescents with ALL can be cured with risk-adjusted and minimal residual disease-guided intensive chemotherapy, without stem cell transplantation. However, some specific subgroups, which are more frequent in adolescents than in children (e.g., early pre-T, iAMP21, and BCR-ABL-like), deserve particular attention. In summary, the advances in treatment of ALL in adolescents have been translated to young adults, and that explains the significant improvement in survival of these patients in recent years.

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