Morphology of myeloproliferative neoplasms

Author:

Ng Zi Yun12,Fuller Kathryn A.1,Mazza‐Parton Allegra1,Erber Wendy N.13ORCID

Affiliation:

1. School of Biomedical Sciences The University of Western Australia Perth Western Australia Australia

2. Haematology Department Royal Perth Hospital Perth Western Australia Australia

3. Department of Haematology PathWest Laboratory Medicine Nedlands Western Australia Australia

Abstract

AbstractMyeloproliferative neoplasms (MPN) are a group of clonal haematological malignancies first described by Dameshek in 1957. The Philadelphia‐negative MPN that will be described are polycythaemia vera (PV), essential thrombocythaemia (ET), pre‐fibrotic myelofibrosis and primary myelofibrosis (PMF). The blood and bone marrow morphology are essential in diagnosis, for WHO classification, establishing a baseline, monitoring response to treatment and identifying changes that may indicate disease progression. The blood film changes may be in any of the cellular elements. The key bone marrow features are architecture and cellularity, relative complement of individual cell types, reticulin content and bony structure. Megakaryocytes are the most abnormal cell and key to classification, as their number, location, size and cytology are all disease‐defining. Reticulin content and grade are integral to assignment of the diagnosis of myelofibrosis. Even with careful assessment of all these features, not all cases fit neatly into the diagnostic entities; there is frequent overlap reflecting the biological disease continuum rather than distinct entities. Notwithstanding this, an accurate morphologic diagnosis in MPN is crucial due to the significant differences in prognosis between different subtypes and the availability of different therapies in the era of novel agents. The distinction between “reactive” and MPN is also not always straightforward and caution needs to be exercised given the prevalence of “triple negative” MPN. Here we describe the morphology of MPN including comments on changes with disease evolution and with treatment.

Publisher

Wiley

Subject

Biochemistry (medical),Clinical Biochemistry,Hematology,General Medicine

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