Molecular determinants of juvenile myelomonosytic leukemia and childhood myelodysplastic syndrome

In the general population the probability of developing cancer before the age of 18 years is around 1 in 400. In the Netherlands, approximately 600 new children each year are diagnosed with cancer (Figure 1). The most common types of childhood cancer are leukemias and the distribution of cancer types varies with age. Figure 2 gives an overview of the incidence of the different subclasses of leukemia as registered by the DCOG (2005-2010). Acute lymphoblastic leukemia (ALL) accounts for about 80% of the leukemias, whereas acute myeloid leukemia (AML) accounts for about 15% of all leukemias. Myelodysplastic syndrome (MDS) and Juvenile Myelomonocytic Leukemia (JMML), which are the subject of this thesis, represent very rare myeloid malignancies in childhood. Over the last decades, following better treatment stratification, better chemotherapy combinations and improved supportive care regimens, the 5-year survival of cancer in children and adolescents improved significantly (Figure 3). The survival of MDS and JMML however, has reached a plateau at approximately 50% following stem cell transplantation, which is the only curative treatment option for these diseases.

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