Primary myelodysplastic syndrome in children

• Published in: Srpski arhiv za celokupno lekarstvo Vol. 129 Suppl 1; p. 10
• Main Authors: Kuzmanović, M, Bunjevacki, G, Rasović, N, Vujić, D, Mićić, D, Guć-Sćekić, M, Jovanović, V
• Format: Journal Article
• Language: Serbian
• Published: Serbia 01-05-2001
• Subjects: Adolescent; Child; Child, Preschool; Female; Humans; Infant; Male; Myelodysplastic Syndromes - blood; Myelodysplastic Syndromes - diagnosis; Myelodysplastic Syndromes - mortality; Prognosis; Survival Rate
• ISSN: 0370-8179

Primary MDS is a group of heterogenous clonal haematopoetic disorders. In a third of patients MDS terminates as acute myeloid leukaemia, usually resisitant to treatment, while the others succumb due to infections and haemorrhage. Conservative managements of MDS (chemotherapy, haematopoetic growth factors, modulation of cytokine network) are unsuccessful, while the bone marrow transplantation is the only definite treatment. We reviewed clinical and haematological presentations, frequency of dysplastic features, histological and cytogenetic findings in 29 children with primary MDS. Indications for haematological evaluation in our patients were symptoms and signs of isolated or combined cytopenias, fever of unknown origin and frequent infections. Hepatosplenomegaly was found in 19 (65%) patients, while this pattern was found in 10% of adult patients. Normochromic anaemia was found in 25 (86%) patients and thrombocytopenia in 23 (76%). Patients presenting pancytopenia had the lowest probability of survival. Degree of dysplasia, histology and kariotype of bone marrow had no influence on survival rates. Prognostic factors in paediatric MDS are of limited significance, as MDS in children is an absolute indication for bone marrow transplantation.