Myeloproliferative Neoplasms and Mastocytosis
This chapter covers the myeloproliferative neoplasms (MPNs) and mastocytosis. The MPNs are clonal neoplasms characterized by increased bone marrow cellularity, organomegaly, and increased effective cellular maturation resulting in elevated blood levels of one or more cell lines (erythrocytosis, leukocytosis, thrombocytosis). Though dysplasia is typically absent, the MPNs can evolve into acute leukemia or an end-stage myelofibrosis. The disease course is typically long, developing over many years. A common theme of tyrosine kinase activation unites the MPNs, including mastocytosis. There is considerable overlap of clinical, laboratory, and pathologic features for many of these neoplasms; over time, one type of MPN may develop features of another. Figures and tables willillustrate the bone marrow pathology of the MPNs and mast cell disease, including relevant clinical, laboratory, cytogenetic, and molecular genetic findings
KeywordsBCR-ABL1 CALR CSF3R MPL JAK2 Chronic myeloid leukemia Essential thrombocythemia Polycythemia vera Primary myelofibrosis Mastocytosis Chronic eosinophilic leukemia, not otherwise specified Chronic neutrophilic leukemia Myeloproliferative neoplasm, unclassifiable
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