The 2008 World Health Organization Classification System for Myeloproliferative Neoplasms Order Out of Chaos

The first formal classification of chronic myeloid neoplasms is credited to William Dameshek, who in 1951 described the concept of myeloproliferative disorders (MPD) by grouping together chronic myelogenous leukemia, polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). The 2001 World Health Organization (WHO) classification of myeloid malignancies included these MPDs under the broader category of chronic myeloproliferative diseases (CMPD), which also included chronic neutrophilic leukemia, chronic eosinophilic leukemia/hypereosinophilic syndrome (CEL/HES), and CMPD, unclassifiable. The revised 2008 WHO classification system featured the following changes: 1) the term CMPD was replaced by myeloproliferative neoplasm (MPN), 2) mast cell disease was formally included under the category of MPN, and 3) the subcategory of CEL/HES was reorganized into CEL not otherwise specified (CEL-NOS) and myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB, and FGFR1; CEL-NOS remained a subcategory of MPN, whereas the latter neoplasms were now assigned a new category of their own. Furthermore, diagnostic criteria for PV, ET, and PMF were revised by incorporating recently described molecular markers (eg, JAK2 and MPL mutations) as well as underscoring the role of histology in differentiating reactive from clonal myeloproliferations. As a result, red cell mass measurement is no longer necessary for the diagnosis of PV, and ET can now be diagnosed at a lower platelet count threshold. The revised WHO document continues to promote the recognition of histologic categories as a necessary first step toward the genetic characterization of myeloid malignancies. Cancer 2009;115:3842-7. (C) 2009 American Cancer Society.