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Curr Opin Hematol. 2018 Mar;25(2):112-119. doi: 10.1097/MOH.0000000000000402.

Polycythemia vera and essential thrombocythemia: algorithmic approach.

Author information

1
Departmente of experimental and clinical medicine, CRIMM, Center Research and Innovation of Myeloproliferative Neoplasms, University of Florence, AOU Careggi, Florence, Italy.
2
Department of Internal Medicine, Divisions of Hematology and Laboratory Medicine, Mayo Clinic, Rochester, Minnesota, USA.

Abstract

PURPOSE OF REVIEW:

To describe an algorithm-based approach, whenever available, to the diagnosis, the risk stratification criteria informing therapy and the current management of polycythemia vera and essential thrombocythemia.

RECENT FINDINGS:

Description of recurrent genetic abnormalities in driver genes, including Janus Kinase 2 (JAK2), myeloproliferative leukemia and calreticulin, a better appreciation of the key diagnostic role of bone marrow features, results of large epidemiologic studies and a few but landmark controlled clinical trials produced in the last decade, all resulted in a reappraisal of the approach to polycythemia vera and essential thrombocythemia.

SUMMARY:

The revised 2017 WHO classification of polycythemia vera and essential thrombocythemia allows early diagnosis and accurate distinction from other chronic myeloproliferative neoplasms, particulary prefibrotic myelofibrosis. The prognostic value of selected mutations is being appreciated and JAK2V617F mutation is currently incorporated as risk variable in prognostic system for essential thrombocythemia. Risk-adjusted stratification is used to select therapeutic approaches that include target agents. However, there is not yet a curative approach to these hematologic neoplasms, and although their management has much improved in the last decades, the associated morbidity and mortality remains significant and may be worsened by toxicities of therapeutic agents. Therefore, several clinically relevant endpoints remain unmet.

PMID:
29194068
DOI:
10.1097/MOH.0000000000000402
[Indexed for MEDLINE]

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