The Incidence,Diagnosis and Treatment of Myelodysplastic Syndrome in Children in the Czech Republic:Results of the Prospective Study of the EWOG-MDS 1998–2002

Overview

The Czech Working Group for Paediatric Haematology participated in the international prospectivestudy for the diagnosis and therapy of myelodysplastic syndrome (MDS) in childhood organized by theEuropean Working Group for Myelodysplastic Syndrome (EWOG-MDS) in 1998–2002. The aim of thestudy was to improve the accuracy of diagnosis in children and adolescents withMDS by a standardizedreview of morphology and standardized cytogenetic and molecular analyses. The secondary objectiveof the study was to improve survival of children with primary MDS over that reported in the literature.During the study period (1998–2002), twenty-one children were diagnosed with MDS in the CzechRepublic – 16 with primary MDS and juvenile myelomonocytic leukaemia (JMML); 3 with myeloidleukaemia and Down’s syndrome; and 2 with secondary MDS. MDS accounted for 5.5–8 % of thehaematologicmalignancies.Of the 16 childrenwith primaryMDS 5 suffered from refractory cytopenia,7 from RAEB/RAEBt, and 4 from JMML. The most common cytogenetic abnormality was monosomy 7in 33 % of the patients. Two boys with primary MDS had a predisposing condition – neurofibromatosistype 1. Thirteen children underwent stem cell transplantation, 4 were treated by intensive AML-typechemotherapy, and 4 patients were followed up without treatment. Eleven children (52 %) are alive andwell.

Key words:
MDS, children, epidemiology, morphology, cytogenetics, stem cell transplantation