Myelodysplastic syndromes in patients under 50 years old: a single institution experience

Breccia, Massimo; Mengarelli, Andrea; Mancini, Marco; Biondo, Francesca; Gentilini, Fabiana; Latagliata, Roberto; Mandelli, Franco; Alimena, Giuliana

doi:10.1016/j.leukres.2005.01.003

« Previous Next »Leukemia Research
Volume 29, Issue 7 , Pages 749-754, July 2005

Myelodysplastic syndromes in patients under 50 years old: a single institution experience

Massimo Breccia
- Department of Cellular Biotechnology and Hematology, University La Sapienza, Rome, Italy
- Corresponding author. Present address: Department of Human Biotechnology and Hematology, Via Benevento 6, 00161 Rome, Italy. Tel.: +39 06 85 79 51; fax: +39 06 44 24 19 84.
Andrea Mengarelli
- Department of Haematology, Regina Elena Cancer Institute, Rome, Italy
Marco Mancini
- Department of Cellular Biotechnology and Hematology, University La Sapienza, Rome, Italy
Francesca Biondo
- Department of Cellular Biotechnology and Hematology, University La Sapienza, Rome, Italy
Fabiana Gentilini
- Department of Cellular Biotechnology and Hematology, University La Sapienza, Rome, Italy
Roberto Latagliata
- Department of Cellular Biotechnology and Hematology, University La Sapienza, Rome, Italy
Franco Mandelli
- Department of Cellular Biotechnology and Hematology, University La Sapienza, Rome, Italy
Giuliana Alimena
- Department of Cellular Biotechnology and Hematology, University La Sapienza, Rome, Italy

Received 23 December 2004; accepted 3 January 2005. published online 07 March 2011.

Abstract

We report on our experience relating to 62 patients with myelodysplastic syndrome (MDS) aged less than 50 years, seen at our Institution and conservatively treated from July 1983 to December 2000. Patients demographics and clinical features at diagnosis were analysed for their prognostic value on survival and on risk of transformation to acute leukaemia.

The median age at diagnosis was 43 years (range 21–50). According to FAB criteria there were 30 patients with refractory anaemia (RA), 3 with refractory anaemia with ringed sideroblasts (RARS), 18 with refractory anaemia with excess of blasts (RAEB), 6 with refractory anaemia with excess of blasts in transformation (RAEB-t) and 5 with chronic myelomonocytic leukaemia (CMML). Fifty patients had evaluable cytogenetic analysis: the most frequent karyotypic change was trisomy of chromosome 8 (10%), followed by monosomy 7 (6%); partial chromosome deletions and translocations were also common abnormalities, occurring on the whole in 16% of patients. At a median follow-up of 15 months 19 patients (31%) progressed to acute myeloid leukaemia (AML).

From univariate analysis we identified some features, which appeared to be predictive of outcome and risk of transformation to AML. Age above 40 years (p=0.002) and high risk according to IPSS score (p=0.002) were found to be predictive for a shorter survival; FAB grouping (p=0.0001), percentage >5% of blasts in the bone marrow (p=0.001) and high risk by IPSS score (p=0.0003) were found to be predictive for a higher risk of transformation to AML.