AI Article Synopsis
- - Advanced myelodysplastic syndrome (MDS) in children is classified into two types: refractory anaemia with excess blasts (RAEB) and RAEB in transformation (RAEB-T), requiring careful diagnosis using clinical features and cytogenetics rather than just blast counts to distinguish it from acute myeloid leukaemia (AML).
- - The most common cytogenetic change in pediatric MDS is monosomy 7, which doesn’t significantly affect prognosis, while those with complex karyotypes generally face a very poor outcome.
- - The best treatment option is haematopoietic stem cell transplantation (HSCT), which has around a 60% cure rate; however, intensive chemotherapy prior to HSCT isn
Article Abstract
Advanced myelodysplastic syndrome (MDS) in children includes refractory anaemia with excess blasts (RAEB) and RAEB in transformation (RAEB-T) according to the paediatric modification of the World Health Organization classification. Clinical features and cytogenetics are essential to make a diagnosis because blast count alone is insufficient to differentiate MDS from acute myeloid leukaemia (AML). Little is known about molecular genetics in paediatric MDS but hypermethylation seem to be frequent. Monosomy 7 is the most common cytogenetic aberration but prognostic neutral whereas those with structural complex karyotype have a very poor outcome. Haematopoietic stem cell transplantation (HSCT) is the treatment of choice and results in cure rates of around 60%. Intensive chemotherapy prior to HSCT provides no survival benefit for children with RAEB and RAEB-T and can generally not be recommended. Intensive chemotherapy before HSCT should be considered in patients with myelodysplasia-related-AML (MDR-AML).
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| http://dx.doi.org/10.1111/j.1365-2141.2011.08724.x | DOI Listing |
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