Myelodysplastic/myeloproliferative neoplasms: a disease in need of recognition


Myelodysplastic/myeloproliferative neoplasms (MDS/MPN) represent a unique subset of hematopoietic stem cell tumors characterized by clinical, morphologic and laboratory features of both MDS and MPN. According to the 2008 WHO classification, these myeloid neoplasms include four hematopathologic entities, namely chronic myelomonocytic leukemia (CMML), BCR–ABL1-negative atypical chronic myeloid leukemia, juvenile myelomonocytic leukemia (JMML) and MDS/MPNs unclassifiable (MDS/MPN-U) as well as a provisional entity of refractory anemia with ring sideroblasts and thrombocytosis (RARS-T). CMML, the most common MDS/MPN is characterized by the presence of a persistent monocytosis (monocyte count >1000 / μl and monocytes comprising >10% of the absolute white blood cell count). JMML is more frequent in pediatric populations and is associated with neurofibromatosis-1. These patients also exhibit monocytosis. Patients with atypical chronic myeloid leukemia manifest neutrophilia with a left shift, significant dysgranulocytosis and an absence of both monocytosis and the BCR–ABL1 translocation, differentiating this entity from CMML and chronic myeloid leukemia.

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