Management of myeloproliferative neoplasms: From academic guidelines to clinical practice

Giovanni Barosi, Letizia Lupo, Vittorio Rosti

Research output: Contribution to journalArticle

16 Citations (Scopus)

Abstract

Myeloproliferative neoplasms (MPNs) are clonal disorders characterized by excessive production of mature cells. In most of the classic Philadelphia-negative MPNs-polycythemia vera (PV), essential thrombocythemia (ET), and MPN-associated myelofibrosis (MPN-MF)-oncogenic mutations affecting JAK2 or MPL lead to constitutive activation of cytokine-regulated intracellular signalling pathways. The traditional therapy for PV and ET is the prevention of thrombotic events with antiproliferative agents in association with aspirin. New drugs such as pegylated interferon and anti-JAK agents are candidates for slowing the evolution to myelofibrosis or leukemia. Conventional therapy for MPN-MF is driven by clinical needs, primarily anemia and splenomegaly. Lenalidomide and pomalidomide are new candidates for treating anemia. JAK2 ATP-competitive inhibitors or drugs that indirectly inhibit the JAK-STAT pathway, like RAD001, are the new candidates for splenomegaly in MPN-MF, but in spite of their strong rationale, they have shown only a palliative benefit. Allogeneic stem cell transplantation remains the only potentially curative approach.

Original languageEnglish
Pages (from-to)50-56
Number of pages7
JournalCurrent Hematologic Malignancy Reports
Volume7
Issue number1
DOIs
Publication statusPublished - Mar 2012

Fingerprint

Guidelines
Essential Thrombocythemia
Polycythemia Vera
Primary Myelofibrosis
Splenomegaly
Neoplasms
Anemia
Stem Cell Transplantation
Pharmaceutical Preparations
Interferons
Aspirin
Leukemia
Adenosine Triphosphate
Cytokines
Mutation
Therapeutics

Keywords

  • Epigenetic drugs
  • Essential thrombocythemia
  • Everolimus
  • JAK inhibitors
  • JAK2 V617F
  • Lenalidomide
  • Myelofibrosis
  • Myeloproliferative neoplasms
  • Polycythemia vera
  • Pomalidomide
  • Stem cell
  • Thalidomide

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Cancer Research

Cite this

Management of myeloproliferative neoplasms : From academic guidelines to clinical practice. / Barosi, Giovanni; Lupo, Letizia; Rosti, Vittorio.

In: Current Hematologic Malignancy Reports, Vol. 7, No. 1, 03.2012, p. 50-56.

Research output: Contribution to journalArticle

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