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9 - Grade/differentiation unknown, not stated, or not applicable
MDS with 5q deletion
Myelodysplastic syndrome with 5q deletion (5q-syndrome)
Bone marrow dysplasia showing refractory macrocytic anemia with oval macrocytes and nonlobulated megakaryocytes. 75% of patients have increased cellularity of marrow. One third of patients have chromosomal abnormality that consists of deletion of the long arm of chromosome 5 (5q). Marrow blasts <5%. Platelet count normal to elevated No Auer rods Normal to increased megakaryocytes
MDS with associated del(5q) is an MDS involves that blood and bone marrow. The most common symptoms are usually related to anemia which is often severe and usually macrocytic. Thrombocytosis is present in up to one half of patients while thrombocytopenia is uncommon. This disease is associated with a median survival of 145 months with transformation to acute myeloid leukemia occurring in less than ten percent of patients. Recently the thalidomide analog lenalidomide has been shown to benefit MDS patients. Transfusion independence was achieved in two-thirds of patients.
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.