Data from the Registry of the Patients with Myelodysplastic Syndrome of a Romanian Single Center. III Analysis of the Group under 50 Years Old.

Abstract

Introduction. The myelodysplastic syndrome (MDS) is mostly observed in patients older than 65 years and therefore there are few reports refering to patients less than 50 years old. However, the interest for this group of MDS patients is increasing because: these patients are doing an active life and therefore the disease have higher socio-economic consequences, their comorbidities are much more rare, there are important ethnic differences in frequency between western and eastern countries, the age-related genetic instability could not be incriminated in the pathogenesis of their MDS, they are the main candidates for aggressive treatments (high doses chemotherapy, bone marrow transplantation).Patients and methods. Sixty six cases with age under 50 years were extracted from the data-base of the MDS Registry of the Clinic of Hematology, Fundeni Clinical Institute, Bucharest, Romania comprising 404 primary cases, collected between 1982 and 2004. The registration form, using the FAB classification, was kindly provided by MDS Foundation (USA)(Chairman Prof. J.M. Bennett). The parameters included in the analysis were: age at presentation, sex, place of residence, values of hemoglobin, neutrophil count, platelet count, percentage of bone marrow blasts, prognostic scores, duration until acute leukemia (AL) transformation. The frequency of different subtypes of MDS and the dynamics of the new cases during the analysed period were also determined. A comparison with the group of age above 60 years and with other similar reference studies was performed.Results. There were 66 (16,7%) cases from which 22 (62,6%) were under 40 and 19 (28.3%) under 30 years old, with a mean age of 34,7 years. A global predominance of the feminine gender and of the urban location with no geographic aggregation could be noticed. The mean values of hemoglobin, neutrophils and platelets were 7.6 g/dL, 2,500/μL and 142.000/μL, respectively. Patients with refractory anemia (RA) and refractory anemia with ringed sideroblasts (RARS) accounted for 46.7% of all cases (RA 34,8%, RARS 11,9%), refractory anemia with excess of blasts (RAEB) 20,8%, RAEB in transformation (RAEB-T) 13,4%, chronic myelomonocytic leukemia 4,4% and unclassified 13,4%. The annual number of new cases increased three times during the analysed period, the increase being not uniform, with a peak in 2000, and not homogeneous. The subtypes with the most important increase in time were RA and RAEB-T. The AL transformation could be registered in 25.7%, after a mean time of 5 months.Conclusions. This study indicates a higher proportion (16.7%) and a lower age (34.7) of young patients with MDS in Romania, considered almost in the middle among those reported for western and eastern countries. The tendancy of the annual frequency of the new cases indicated a three times increase. An urban predominant location, in contrast with that of the group above 60 years old, has been noticed. The female predominance appears as a characteristic feature of the patients with MDS from this group of age. The degree of anemia was obviously more severe than that reported in other studies.