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Chronic Myeloproliferative Neoplasms (Other Than Chronic Myeloid Leukemia)
Book chapter

Chronic Myeloproliferative Neoplasms (Other Than Chronic Myeloid Leukemia)

Matthew E Keeney and Sharathkumar Bhagavathi
Practical Lymph Node and Bone Marrow Pathology, pp.517-530
Practical Anatomic Pathology, Springer International Publishing
03/29/2020
DOI: 10.1007/978-3-030-32189-5_23

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Abstract

The myeloproliferative neoplasms (MPNs) are a group of leukemias that result from clonal expansion of one or more myeloid lineage cells. This clonal expansion is due to mutant tyrosine kinases or mutated downstream proteins. The related molecular drivers cause overlapping pathological, laboratory, and clinical presentations. Some MPNs share oncogenic driver mutations, while others are defined by specific translocations. The accurate diagnosis has prognostic and therapeutic implications and requires close correlation with pathological, clinical, and laboratory data. Most MPNs are initially indolent with effective hematopoiesis early in the course of disease; however, they typically progress to an acute leukemia, a marrow failure due to myelofibrosis, or a myelodysplastic syndrome. This leads to a wide variation of morphologic appearances for the pathologist depending on when in the patient’s natural course of disease the sample crosses the microscope stage.
Atypical megakaryocytes Chronic leukemia Eosinophilia Mastocytosis Mutant tyrosine kinase Myelofibrosis Myeloproliferative Panmyelosis Ruxolitinib

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