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New classification for myelodysplasia
In order to properly treat myelodysplasia syndrome, Down syndrome–related diseases have been reclassified as a separate entity.
Sharon M Castellino, MD, FAAP Wake Forest University Health Sciences
Timothy P Cripe, MD, PhD Cincinnati Children’s Hospital Medical Center
Scott C Howard, MD Cincinnati Children’s Hospital Medical Center
The myelodysplasia syndromes (MDS) are clonal stem cell disorders characterized by progressive cytopenia or cytopenias, usually in the presence of a hypercellular bone marrow and multilineage dysplasia. Usually, all 3 cell lines (myeloid/monocyte, erythroid, megakaryocyte) are involved. Myelodysplasia syndrome is rare in childhood, and most children have a rapidly progressive course. Myelodysplasia disorders have been defined by their predilection to evolve into acute myeloid leukemias (AML), yet not all cases terminate in leukemia.
For information on Shwachman-Diamond Syndrome check out Shwachman-Diamond America