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9 - Grade/differentiation unknown, not stated, or not applicable
Childhood myelodysplastic syndrome
Refractory cytopenia of childhood
Myelodysplastic syndrome with bi-cytopenia or pancytopenia and dyplastic changes in 10% or more of cells in 2 or more of myeloid cell lines. < 1% blasts in blood, < 5% blasts in marrow, <15% ringed sideroblasts (If > 15%, case should be classified as subtype RCMD-RS) Auer rods not seen, Monocytes in blood < 1 x 109/L
Blood and bone marrow are always involved. Most patients present with evidence of bone marrow failure with cytopenia of two or more myeloid lines. At least 2 types of blood counts are low and have an abnormal appearance under the microscope (dysplasia). The number of blasts is less than 5%. About 25% of people with MDS have this type. It will change into leukemia in about 10% of patients. Having this type of MDS will shorten a person's survival. One estimate is that half of patients will die within 2 years of diagnosis.
This histology code also includes childhod MDS. MDS is very rare in children. Both the peripheral blood and bone marrow are involved. Hepatosplenomegaly is generally not a feature of RCC and lymph nodes are not the initial site of involvement.
MDS is a group name for a number of specific diseases. As the MDS progresses, it may manifest as several subtypes. This is a part of the disease process. Abstracting each of the subtypes would result in over-counting these diseases. Code the first subtype that is diagnosed. Do not change the histology code or create a new abstract for any subsequent specific MDS subtypes.