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Review
. 2024 Feb;65(2):158-167.
doi: 10.1080/10428194.2023.2277674. Epub 2024 Jan 24.

Understanding triple negative myeloproliferative neoplasms: pathogenesis, clinical features, and management

Serena Tharakan  1 , John Mascarenhas  1 , Douglas Tremblay  1
Affiliations
Review

Understanding triple negative myeloproliferative neoplasms: pathogenesis, clinical features, and management

Serena Tharakan et al. Leuk Lymphoma. 2024 Feb.

Abstract

ABSTRACTMyeloproliferative neoplasms (MPNs) that lack the classical "driver mutations," termed triple negative MPNs, remain a poorly understood entity. Despite considerable progress toward understanding MPN pathobiology, the mechanisms leading to the development of these MPNs remains inadequately elucidated. While triple negative primary myelofibrosis (TN-PMF) portends a poor prognosis, triple negative essential thrombocythemia (TN-ET) is more favorable as compared with JAK2 mutated ET. In this review, we summarize the clinical features and prognosis of TN-PMF and -ET as well as diagnostic challenges including identification of non-canonical driver mutations. We also discuss additional molecular drivers to better understand possible pathogenic mechanisms underlying triple negative MPNs. Finally, we highlight current therapeutic approaches as well as novel targets, particularly in the difficult to treat TN-PMF population.

Keywords: JAK2; KRAS; Myelofibrosis; essential thrombocythemia; triple negative.

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