Primary Myelofibrosis

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

This chapter illustrates the diagnosis and treatment of primary myelofibrosis (PMF), a myeloproliferative neoplasm (MPN). PMF is diagnosed according to a 2008 World Health Organization system. This disorder is characterized by stem cell-derived clonal myeloproliferation and the presence of somatic mutations involving JAK2 (in the majority of patients) and MPL or other somatic mutations (in the minority). Current prognostication in PMF is best accomplished by the use of the International Prognostic Scoring System (IPSS), dynamic IPSS (DIPSS), or DIPSS-plus. High-risk or intermediate-2 risk patients with PMF should always be considered for either investigational drug therapy or allogeneic hematopoietic cell transplant. Ruxolitinib, a JAK1-JAK2 inhibitor, is used in patients with high-or intermediate-risk disease. In general, allogeneic stem cell transplant is a treatment modality whose benefits should be carefully balanced against its risks. Pomalidomide, other JAK-inhibiting adenosine triphosphate (ATP) mimetics, and other novel drugs (e.g., mTOR inhibitors) are currently being tested in patients with MF.

Original languageEnglish (US)
Title of host publicationCancer Consult: Expertise for Clinical Practice
PublisherWiley Blackwell
Pages162-166
Number of pages5
ISBN (Print)9781118589199, 9781118589212
DOIs
StatePublished - Jun 20 2014

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Keywords

  • DIPSS-plus
  • Dynamic IPSS (DIPSS)
  • International Prognostic Scoring System (IPSS)
  • Myeloproliferative neoplasm (MPN)
  • Primary myelofibrosis (PMF)

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Tefferi, A. (2014). Primary Myelofibrosis. In Cancer Consult: Expertise for Clinical Practice (pp. 162-166). Wiley Blackwell. https://doi.org/10.1002/9781118589199.ch26