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Clinical Features and Prognostic Assessment in 233 Patients with Therapy-Related Myelodysplastic Syndromes: The IPSS-R Is a Powerful Predictor of Outcome

Authors :
María Teresa Ardanaz
Benet Nomdedeu
María López-Pavía
Dolors Costa
Guillermo Sanz
Arturo Pereira
Carmen Pedro
Xavier Calvo
María Teresa Cedena
Jordi Esteve
Montserrat Martínez de Sola
Brayan Marcel Merchán
Meritxell Nomdedeu
Ana A. Martín
Francesc Cobo
Source :
Blood. 124:4636-4636
Publication Year :
2014
Publisher :
American Society of Hematology, 2014.

Abstract

Background Therapy-related Myelodysplastic Syndromes (t-MDS) are those MDS occurring after cytotoxic and/or radiation therapy administered for a prior neoplastic or non-neoplastic disorder. Their prognosis is generally very poor. The commonly used risk prognostic models for MDS (IPSS and IPSS-R) are not validated in this entity as they were developed after the exclusion of therapy-related cases (Greenberg et al. Blood 1997; Greenberg et al. Blood 2012). Aims The main aims of this study are: a) to report clinical findings and overall survival on 233 patients with t-MDS, and to compare them with a large series of de novo cases; b) to test if IPSS-R is applicable to t-MDS patients. Patients and methods The study is based on the Spanish Registry for MDS, a retrospective database that includes more than 10000 cases. The investigators were asked to fill in a questionnaire regarding prior disease (PD) and prior therapy in those cases reported to be t-MDS. Herein are described the clinical features and overall survival of the first 233 cases with the required information, and compared with patients with de novo MDS from a single center series (n=725). Log Rank test was applied to asses IPSS-R in t-MDS group. Results The 233 reported patients were diagnosed between January 1993 and February 2014. The series includes 104 women (44,6%) and 129 men (55,4%). One hundred and two patients (43.9%) had a primary hematologic malignancy, 119 (51%) had a solid tumor, and 12 (5.1%) received cytotoxic therapy for autoimmune disorders. Ninety eight patients (42.6%) received only chemotherapy (CT), 45 (19.6%) received only radiotherapy (RT), 44 (19.1%) received combined modality treatment (CMT), and 43 (18.6%) received an autologous stem cell transplantation (ASCT). The median time of latency between PD and diagnosis in t-MDS group was 4.56 years (range: 0.03-29.63) in patients previously treated with CT or CMT, significantly lower than the observed after RT (8.54; range 0.83-23.02) or ASCT (8.64; range 2.87-28.32) groups (p=0.023 and p Conclusions In conclusion, our data supports the notion that t-MDS may contain entities with heterogeneous prognosis due to a diverse biological basis. The capacity of the IPSS-R for separating good prognostic from bad prognostic cases shown in this study could be very useful in the clinical setting in order to offer risk-adapted treatments to patients, although the development of a specific prognostic score for these entities would be needed. Disclosures No relevant conflicts of interest to declare.

Details

ISSN :
15280020 and 00064971
Volume :
124
Database :
OpenAIRE
Journal :
Blood
Accession number :
edsair.doi...........4c3a0d256d7e75526d77d934ba72186d
Full Text :
https://doi.org/10.1182/blood.v124.21.4636.4636