SEER is an authoritative source of information on cancer incidence and survival in the United States. SEER currently collects and publishes cancer incidence and survival data from population-based cancer registries covering approximately 28 percent of the U.S. population.
Acute myeloidleukemia in DS is usually an acute megakaryoblastic leukemia. There are no biological differences in Down Syndrome's individuals between myelodysplastic syndrome (MDS) and AML. In the preleukemia phase, the disease has the features of Refractory cytopenia of childhood (RCC).
Individuals with DS have a 50x increase in incidence of acute leukemia during the first 5 years of life compared to non-DS individuals. This is part of the myeloid proliferations with Down syndrome WHO group, which also includes transient abnormal myelopoiesis (see 9898/1).
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010 see code 9860/3.)
This disease is unique to children with Down's Syndrome (DS). Blood and bone marrow are the principle sites of involvement. Extramedullary involvement, mainly of spleen and liver, is almost always present.
There is no biological differences between myelodysplastic syndrome (MDS) and overt acute myeloidleukemia (AML) in DS individuals. Because this type of disease is unique to children with DS, the term Myeloidleukemia (AML) of DS encompasses both neoplasms, MDS and AML.