Abstract

This review highlights the main changes in the revised 2016 WHO classification of myeloid neoplasms (published in 2017) that impact diagnosis and ultimately impact management of patients with these diseases. The revision was based on data accumulated since the 2008 WHO classification, much of which relate to new molecular genetic information about these neoplasms. This massive recent influx of data concerning the significance of pathogenic mutations has affected all myeloid neoplasm categories. The new information has been incorporated as part of the diagnostic criteria of many diseases and has led to the creation of new provisional entities defined by genetic features. Germline mutations that predispose to myeloid neoplasms are also emerging as important findings that impact disease classification. The growing body of genetic data have not only altered the classification of myeloid neoplasms, but are also impacting patient management. Genetically-defined disease categories have characteristic prognoses and predicted clinical behavior. Some mutations are associated with responsiveness to certain therapies, including those that target relevant oncogenes. The disease categories in the new classification facilitate the application of risk-adapted therapy based on the most recently available data.

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