Childhood myelodysplastic syndromes (MDS) are a heterogeneous group of stem cell disorders characterized by peripheral blood cytopenia, ineffective hematopoiesis, hyper- or hypocellular bone marrow (BM) and propensity to evolve into acute myeloid leukemia (AML) in approximately 30–40% of cases.1, 2, 3 Childhood MDS are rare diseases and several differences between adult and pediatric MDS have been recognized.1 MDS in pediatric patients is classified, using morphological criteria, into refractory cytopenia of childhood (RCC), refractory anemia with excess of blasts (RAEB) and RAEB in transformation (RAEB-t). Different from adult MDS classification according to World Health Organization (WHO) indications, pediatric MDS patients with a blast count of 20–30% are classified as MDS and not as AML. As relative blasts count alone is not sufficient to differentiate AML from MDS, morphological evaluation remains crucial for diagnosis of MDS.1
Gene expression signatures of pediatric myelodysplastic syndromes are associated with risk of evolution into acute myeloid leukemia
BRESOLIN, SILVIA;TRENTIN, LIVIO;BASSO, GIUSEPPE;TE KRONNIE, GEERTRUDY;
2012
Abstract
Childhood myelodysplastic syndromes (MDS) are a heterogeneous group of stem cell disorders characterized by peripheral blood cytopenia, ineffective hematopoiesis, hyper- or hypocellular bone marrow (BM) and propensity to evolve into acute myeloid leukemia (AML) in approximately 30–40% of cases.1, 2, 3 Childhood MDS are rare diseases and several differences between adult and pediatric MDS have been recognized.1 MDS in pediatric patients is classified, using morphological criteria, into refractory cytopenia of childhood (RCC), refractory anemia with excess of blasts (RAEB) and RAEB in transformation (RAEB-t). Different from adult MDS classification according to World Health Organization (WHO) indications, pediatric MDS patients with a blast count of 20–30% are classified as MDS and not as AML. As relative blasts count alone is not sufficient to differentiate AML from MDS, morphological evaluation remains crucial for diagnosis of MDS.1Pubblicazioni consigliate
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