ABSTRACT
Myeloproliferative neoplasms (MPNs; Table 28.1) represent a heterogeneous range of clonal hematopoietic stem-cell diseases that can be subdivided into classic and nonclassic myeloproliferative disorders. The first group includes chronic myeloid leukemia (CML) and three major non-CML categories: polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). Nonclassic MPNs include chronic neutrophilic leukemia (CNL) and chronic eosinophilic leukemia (CEL). Systemic mastocytosis (SM) and myeloid/lymphoid neoplasms with eosinophilia and rearrangement of platelet-derived growth factor receptor α (PDGFRA), platelet-derived growth factor receptor β (PDGFRB), or FGFR1, or with PCM1-JAK2 are not considered a subgroup of MPN in the revised 2016 WHO classification and are now separate categories [1,2]. Simplified diagnostic workup for MPN is presented in Figure 28.1.
