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Current Hematologic Malignancy Reports

, Volume 13, Issue 3, pp 173–182 | Cite as

Post-ET and Post-PV Myelofibrosis: Updates on a Distinct Prognosis from Primary Myelofibrosis

  • Francesco PassamontiEmail author
  • Barbara Mora
  • Daniela Barraco
  • Margherita Maffioli
Myeloproliferative Neoplasms (B Stein, Section Editor)
  • 607 Downloads
Part of the following topical collections:
  1. Topical Collection on Myeloproliferative Neoplasms

Abstract

Purpose of Review

The purpose of this review is to help doctors in the management of patients with post-polycythemia (PPV) and post-essential thrombocythemia (PET) myelofibrosis (MF) facing diagnostic criteria, prognostication, and treatment possibilities.

Recent Findings

Diagnostic criteria of primary myelofibrosis (PMF) have been recently updated from the WHO classification. A clear-cut distinction between pre-fibrotic and overt PMF has been done. Concerning PPV and PET MF, the criteria come from 2008. Prognostication of PMF has been well established on clinical criteria, but recent molecular acquisitions will improve the strategy. For PPV and PET MF, the new MYSEC-PM is helpful for prediction of survival. JAK2-inhibitors and stem cell transplant are the two critical therapeutic approaches in myelofibrosis.

Summary

Differences between PMF and SMF substantiate the efforts underway to adequately stratify SMF patients with ad hoc prognostic tools and to use such categorization to evaluate available treatment modalities.

Keywords

Polycythemia Thrombocythemia Myelofibrosis Prognosis JAK2 Survival 

Notes

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Authors and Affiliations

  • Francesco Passamonti
    • 1
    • 2
    • 3
    Email author
  • Barbara Mora
    • 1
  • Daniela Barraco
    • 1
  • Margherita Maffioli
    • 1
  1. 1.Department of Medicine and SurgeryUniversity of InsubriaVareseItaly
  2. 2.Hematology, Department of Specialistic MedicineOspedale di Circolo, ASST Sette LaghiVareseItaly
  3. 3.Hematology, Dipartimento di Medicina e ChirurgiaUniversity of InsubriaVareseItaly

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