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Philadelphia chromo...
Philadelphia chromosome-negative classical myeloproliferative neoplasms : revised management recommendations from European LeukemiaNet
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- Barbuil, Tiziano (author)
- Osped Giovanni 23, Div Hematol, Bergamo, Italy
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- Tefferi, Ayalew (author)
- Mayo Clin, Dept Med, Div Hematol, Rochester, MN USA
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- Vannucchi, Alessandro M. (author)
- Univ Florence, AOU Careggi, Ctr Res & Innovat Myeloproliferat Neoplasms, CRIMM, Florence, Italy
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- Passamonti, Francesco (author)
- Univ Insubria, Osped Circolo, Dept Med & Surg, Div Hematol,ASST Sette Laghi, Varese, Italy
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- Silvers, Richard T. (author)
- Weill Cornell Med, Div Hematol Oncol, New York, NY USA
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- Hoffman, Ronald (author)
- Mt Sinai Sch Med, Dept Med, Tisch Canc Inst, New York, NY USA
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- Verstovsek, Srdan (author)
- Univ Texas MD Anderson Canc Ctr, Dept Leukemia, Houston, TX 77030 USA
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- Mesa, Ruben (author)
- UT Hlth San Antonio Canc Ctr, San Antonio, TX USA
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- Kiladjian, Jean-Jacques (author)
- Univ Paris 07, Hop St Louis, AP HP, INSERM,Ctr Invest Clin CIC 1427, Paris, France
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- Hehlmann, Rudiger (author)
- Heidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany
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- Reiter, Andreas (author)
- Heidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany
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- Cervantes, Francisco (author)
- Univ Barcelona, IDIBAPS, Hosp Clin, Barcelona, Spain
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- Harrison, Claire (author)
- Guys & St Thomas NHS Fdn Trust, Dept Hematol, London, England
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- Mc Mullin, Mary Frances (author)
- Queens Univ, Ctr Med Educ, Belfast, Antrim, North Ireland
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- Hasselbalch, Hans Carl (author)
- Zealand Univ Hosp, Dept Hematol, Roskilde, Denmark
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- Koschmieder, Steffen (author)
- Rhein Westfal TH Aachen, Fac Med, Dept Hematol Oncol Hemostaseol & Stem Cell Transp, Aachen, Germany
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- Marchetti, Monia (author)
- Hosp Cardinal Massaia, Oncol SOC, Hematol Day Serv, Asti, Italy
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- Bacigalupo, Andrea (author)
- Univ Cattolica Sacro Cuore, Fdn Policlin Univ Gemelli, Ist Ematol, Rome, Italy
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- Finazzil, Guido (author)
- Osped Giovanni 23, Div Hematol, Bergamo, Italy
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- Kroeger, Nicolaus (author)
- Univ Hosp Hamburg Eppendorf, Dept Stem Cell Transplantat, Hamburg, Germany
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- Griesshammer, Martin (author)
- Univ Hannover, Acad Hosp, Johannes Wesling Med Ctr Minden, Dept Hematol & Oncol, Minden, Germany
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- Birgegård, Gunnar, 1944- (author)
- Uppsala universitet,Hematologi
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- Barosi, Giovanni (author)
- IRCCS Policlin S Matteo Fdn, Ctr Study Myelofibrosis, Pavia, Italy
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(creator_code:org_t)
- 2018-02-27
- 2018
- English.
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In: Leukemia. - : Nature Publishing Group. - 0887-6924 .- 1476-5551. ; 32:5, s. 1057-1069
- Related links:
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https://europepmc.or...
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https://urn.kb.se/re...
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https://doi.org/10.1...
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Abstract
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- 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.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Cancer och onkologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Cancer and Oncology (hsv//eng)
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Barbuil, Tiziano
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Tefferi, Ayalew
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Vannucchi, Aless ...
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Passamonti, Fran ...
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Silvers, Richard ...
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Hoffman, Ronald
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Verstovsek, Srda ...
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Mesa, Ruben
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Kiladjian, Jean- ...
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Hehlmann, Rudige ...
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Reiter, Andreas
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Cervantes, Franc ...
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Harrison, Claire
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Mc Mullin, Mary ...
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Hasselbalch, Han ...
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Koschmieder, Ste ...
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Marchetti, Monia
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Bacigalupo, Andr ...
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Finazzil, Guido
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Kroeger, Nicolau ...
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Griesshammer, Ma ...
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Birgegård, Gunna ...
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Barosi, Giovanni
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Leukemia
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Uppsala University