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Leukemia. 2018 May;32(5):1057-1069. doi: 10.1038/s41375-018-0077-1. Epub 2018 Feb 27.

Philadelphia chromosome-negative classical myeloproliferative neoplasms: revised management recommendations from European LeukemiaNet.

Author information

1
Division of Hematology, Ospedale Giovanni XXIII, Bergamo, Italy.
2
Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN, USA.
3
CRIMM, Center Research and Innovation of Myeloproliferative Neoplasms, University of Florence, AOU Careggi, Florence, Italy.
4
Division of Hematology, Department of Medicine and Surgery, Ospedale di Circolo, University of Insubria, ASST Sette Laghi, Varese, Italy.
5
Division of Hematology-Oncology, Weill Cornell Medicine, New York, NY, USA.
6
Tisch Cancer Institute, Department of Medicine, Mount Sinai School of Medicine, New York, NY, USA.
7
Department of Leukemia, M.D. Anderson Cancer Center, University of Texas, Houston, TX, USA.
8
UT Health San Antonio Cancer Center, San Antonio, TX, USA.
9
Centre d'Investigations Cliniques (CIC 1427), Assistance Publique-Hôpitaux de Paris, Hôpital Saint-Louis, INSERM, Université Paris 7, Paris, France.
10
Department of Hematology and Oncology, University Hospital Mannheim, Heidelberg University, Mannheim, Germany.
11
Hospital Clínic, IDIBAPS, University of Barcelona, Barcelona, Spain.
12
Department of Hematology, Guy's and St. Thomas' NHS Foundation Trust, London, UK.
13
Centre for Medical Education, Queen's University, Belfast, UK.
14
Department of Hematology, Zealand University Hospital, Roskilde, Denmark.
15
Department of Hematology, Oncology, Hemostaseology and Stem Cell Transplantation, Faculty of Medicine, RWTH Aachen University, Aachen, Germany.
16
Hematology Day Service, Oncology SOC, Hospital Cardinal Massaia, Asti, Italy.
17
Istituto di Ematologia, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario Gemelli, Rome, Italy.
18
Department of Stem Cell Transplantation, University Hospital Hamburg-Eppendorf, Hamburg, Germany.
19
Department of Hematology and Oncology, Johannes Wesling Medical Centre Minden, Academic Hospital of the University of Hannover, Minden, Germany.
20
Department of Hematology, Uppsala University, Uppsala, Sweden.
21
Center for the Study of Myelofibrosis, IRCCS Policlinico S. Matteo Foundation, Pavia, Italy. barosig@smatteo.pv.it.

Abstract

This document updates the recommendations on the management of Philadelphia chromosome-negative myeloproliferative neoplasms (Ph-neg MPNs) published in 2011 by the European LeukemiaNet (ELN) consortium. Recommendations were produced by multiple-step formalized procedures of group discussion. A critical appraisal of evidence by using Grades of Recommendation, Assessment, Development and Evaluation (GRADE) methodology was performed in the areas where at least one randomized clinical trial was published. Seven randomized controlled trials provided the evidence base; earlier phase trials also informed recommendation development. Key differences from the 2011 diagnostic recommendations included: lower threshold values for hemoglobin and hematocrit and bone marrow examination for diagnosis of polycythemia vera (PV), according to the revised WHO criteria; the search for complementary clonal markers, such as ASXL1, EZH2, IDH1/IDH2, and SRSF2 for the diagnosis of myelofibrosis (MF) in patients who test negative for JAK2V617, CALR or MPL driver mutations. Regarding key differences of therapy recommendations, both recombinant interferon alpha and the JAK1/JAK2 inhibitor ruxolitinib are recommended as second-line therapies for PV patients who are intolerant or have inadequate response to hydroxyurea. Ruxolitinib is recommended as first-line approach for MF-associated splenomegaly in patients with intermediate-2 or high-risk disease; in case of intermediate-1 disease, ruxolitinib is recommended in highly symptomatic splenomegaly. Allogeneic stem cell transplantation is recommended for transplant-eligible MF patients with high or intermediate-2 risk score. Allogeneic stem cell transplantation is also recommended for transplant-eligible MF patients with intermediate-1 risk score who present with either refractory, transfusion-dependent anemia, blasts in peripheral blood > 2%, adverse cytogenetics, or high-risk mutations. In these situations, the transplant procedure should be performed in a controlled setting.

PMID:
29515238
PMCID:
PMC5986069
[Available on 2019-05-01]
DOI:
10.1038/s41375-018-0077-1
[Indexed for MEDLINE]

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