Polycythemia vera and essential thrombocythemia: Algorithmic approach

Alessandro M. Vannucchi, Paola Guglielmelli, Ayalew Tefferi

Research output: Contribution to journalReview article

3 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)112-119
Number of pages8
JournalCurrent Opinion in Hematology
Volume25
Issue number2
DOIs
StatePublished - Mar 1 2018

Fingerprint

Essential Thrombocythemia
Polycythemia Vera
Janus Kinase 2
Calreticulin
Primary Myelofibrosis
Mutation
Controlled Clinical Trials
Hematologic Neoplasms
Epidemiologic Studies
Early Diagnosis
Leukemia
Therapeutics
Bone Marrow
Morbidity
Mortality
Genes
Neoplasms

Keywords

  • essential thrombocythemia
  • JAK2 mutation
  • polycythemia vera
  • therapy
  • thrombosis

ASJC Scopus subject areas

  • Hematology

Cite this

Polycythemia vera and essential thrombocythemia : Algorithmic approach. / Vannucchi, Alessandro M.; Guglielmelli, Paola; Tefferi, Ayalew.

In: Current Opinion in Hematology, Vol. 25, No. 2, 01.03.2018, p. 112-119.

Research output: Contribution to journalReview article

Vannucchi, Alessandro M. ; Guglielmelli, Paola ; Tefferi, Ayalew. / Polycythemia vera and essential thrombocythemia : Algorithmic approach. In: Current Opinion in Hematology. 2018 ; Vol. 25, No. 2. pp. 112-119.
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