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Code grade specified by pathologist. If no grade specified, code 9
M3 Module 5: PH10
Acute myeloid leukemia associated with Down syndrome
AML associated with DS
Acute myeloid leukemia 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. Acute myeloid leukemia in DS accounts for 50% of cases of acute leukemia in DS individuals. There is no biological differences between myelodysplastic syndrome (MDS) and overt acute myeloid leukemia (AML) in DS individuals. Because this type of disease is unique to children with DS, the term Myeloid leukemia (AML) of DS encompasses both neoplasms, MDS and AML.