Primary myelofibrosis (PMF), post polycythemia vera myelofibrosis (post-PV MF), post essential thrombocythemia myelofibrosis (post-ET MF), blast phase PMF (PMF-BP): Consensus on terminology by the international working group for myelofibrosis research and treatment (IWG-MRT)

Mesa, Ruben A.; Verstovšek, Srđan; Cervantes, Francisco; Barosi, Giovanni; Reilly, John T.; Dupriez, Brigitte; Levine, Ross L.; Bousse-Kerdilès, M. C. Le; Wadleigh, Martha; Campbell, Peter J.; Silver, Richard T.; Vannucchi, Alessandro M.; Deeg, H. Joachim; Gisslinger, Heinz; Thomas, Deborah A.; Odenike, Olatoyosi; Solberg, Lawrence A.; Gotlib, Jason; Hexner, Elizabeth O.; Nimer, Stephen D.; Kantarjian, Hagop M.; Orazi, Attilio; Vardiman, James W.; Thiele, Jüergen; Tefferi, Ayalew

doi:10.1016/j.leukres.2006.12.002

Cited by 281 publications

(181 citation statements)

References 25 publications

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“…Concerning the dynamics of disease process in PMF, the former gold standard for the diagnosis of MMM [6,22,23,89,115,116] or agnogenic myeloid metaplasia (AMM) should be avoided, because these criteria include only the advanced or overt stages of a wide spectrum of clinical and morphological disease manifestations [102]. Particularly, to underscore the fact that overt myelofibrosis is not a necessary diagnostic feature of this MPN category, the term PMF was recommended to the WHO by the International Working Group on Myelofibrosis [117]. In this context, the crucial and often disregarded differentiation from terminal stage PV and ET presenting with findings of MMM/AMM [8,9,21,115] was also taken into account [118].…”

Section: Primary Myelofibrosismentioning

confidence: 99%

Prodromal myeloproliferative neoplasms: The 2008 WHO classification

Kvasnicka

Thiele

2009

American J Hematol

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The concept of prodromal chronic myeloproliferative neoplasms has been endorsed by the WHO classification implicating a stepwise evolution of disease. Histology of the bone marrow (BM) and borderline to mildly expressed clinical features play a pivotal role for diagnosing prefibrotic-early primary myelofibrosis. By lowering the platelet count for essential thrombocythemia and regarding BM morphology, early manifestations are tackled. Pre-polycythemic stages of polycythemia vera with a low hemoglobin level at onset are diagnosed by positive JAK2V617F mutation status, a low erythropoietin value, and characteristic BM features. The revised WHO classification incorporates hematological, morphological, and moleculargenetic parameters to generate a consensus-based working diagnosis. Am. J. Hematol. 85:62-69, 2010. V

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Section: Primary Myelofibrosismentioning

confidence: 99%

Prodromal myeloproliferative neoplasms: The 2008 WHO classification

Kvasnicka

Thiele

2009

American J Hematol

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“…MF can present as a de novo disorder (primary myelofibrosis or PMF) or appear as an evolutive form of a previously known polycythemia vera or essential thrombocythemia (post-PV MF or post-ET MF) [3]. The recent finding of the V617F mutation in the JAK2 gene in a high proportion of MF patients [4] and the MPL mutation in some PMF patients [5] has contributed to a better understanding of the pathogenesis of the disease.…”

Section: Introductionmentioning

confidence: 99%

Efficacy and tolerability of hydroxyurea in the treatment of the hyperproliferative manifestations of myelofibrosis: results in 40 patients

et al. 2010

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Hydroxyurea (HU) is frequently given as treatment for myelofibrosis (MF), but data on its efficacy and tolerability are scarce. The results of HU therapy were evaluated in 40 patients with hyperproliferative manifestations of primary (n= 32), post-polycythemia vera (n= 6) or post-essential thrombocythemia (n= 2) myelofibrosis. Median interval between diagnosis and HU start was 6.2 months (range: 0-141.7). Reasons for treatment were: constitutional symptoms (55%), symptomatic splenomegaly (45%), thrombocytosis (40%), leukocytosis (28%), pruritus (10%), and bone pain (8%). The starting dose was 500 mg/day, subsequently adjusted to the individual efficacy and tolerability.Response was: bone pain 100%, constitutional symptoms 82%, pruritus 50%, splenomegaly 40%, and anemia 12.5%. According to the International Working Group for Myelofibrosis Research and Treatment criteria, clinical improvement was achieved in 16 patients (40%). Median duration of response was 13.2 months (range: 3-126.2). Worsening of the anemia or appearance of pancytopenia were observed in 18 patients, requiring administration of erythropoietin-stimulating agents (n=17) and/or danazol (n=9). Oral or leg ulcers appeared in 5 patients and one had gastrointestinal symptoms. HU is an effective and generally well-tolerated therapy for the hyperproliferative manifestations of MF. The accentuation of the anemia often induced by HU is usually manageable with concomitant treatment.Response to Reviewers: Answers to the comments by reviewer 1:1. Retrospective design of the study. As already stated in the Introduction and in the Discussion, although there is limited bibliographic support for the use of hydroxyurea (HU) in MF, in clinical practice, this drug has been considered for many years (and still is) as the standard therapy for the hyperproliferative manifestations of this disease. Actually, until the recent development of the JAK2 inhibitors, no real alternative therapy to HU was available for comparison. As a result, for the time being, a retrospective study is the only possibility to assess the efficacy and tolerability of HU in MF.Having said this, it must be remarked that the data from the present study have been generated in a single institution, in which the drug was used following uniform rules for the starting dose, the dose adjustments and the use of adjuvant drugs for the treatment of the associated anemia. Moreover, more than 80% of the patients in the study were treated by the same physician, with long-lasting and specific dedication to MF.2. Selection of the patients. As stated in the Patients and Methods, the 40 patients included in the study were the only ones receiving HU among the 157 with MF followed in our institution during the study period. As also stated in the same section, HU was given to these 40 patients exclusively as a treatment for the hyperproliferative manifestations of MF (i.e., constitutional symptoms, symptomatic splenomegaly, pruritus, bone pain, leukocytosis, and thrombocytosis). This means that a patient ...

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“…A recent study proposed new criteria for evaluating patients with MPN in blast phase, which included the evaluation of both MPN and AML. 20,21 Although these criteria have not been validated in large studies yet, they may refine prognostic factors of MPN in blast phase before allo-SCT.…”

Section: Discussionmentioning

confidence: 99%

Allo-SCT for Philadelphia-negative myeloproliferative neoplasms in blast phase: a study from the Societe Française de Greffe de Moelle et de Therapie Cellulaire (SFGM-TC)

Cahu

Chevallier

Clavert

et al. 2014

Bone Marrow Transplant

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Progression of Philadelphia-negative myeloproliferative (MPN) or myelodysplastic/myeloproliferative neoplasms (MDS/MPN) to acute myeloid leukemia (AML) is an adverse event in the course of the disease. Although allogeneic hematopoietic SCT (allo-SCT) is considered as the only curative therapy, few data exist on the outcome of patients with Philadelphia-negative MPN or MDS/MPN in blast phase who received an allo-SCT. Sixty patients were included in this retrospective study. AML was secondary to an MPN in 43 cases, whereas AML evolved from an MDS/MPN in 17 cases. Patients received allo-SCT in CR or advanced disease in 26 cases and 34 cases, respectively. With a median follow-up of 31 months (range, 25-44), OS and leukemia-free survival (LFS) were, respectively, 18% and 9% at 3 years. CR at transplant was associated with an improved LFS in univariate and multivariate analysis. The 3-year LFS was 18% for patients undergoing allo-SCT in CR versus 3% in advanced disease (P = 0.008). Absence of thrombosis and an intermediate or favorable AML karyotype were associated with an improved outcome for patients who received allo-SCT in CR. New strategies are needed to improve the outcome of patients with MPN-MDS/MPN in blast phase.

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Primary myelofibrosis (PMF), post polycythemia vera myelofibrosis (post-PV MF), post essential thrombocythemia myelofibrosis (post-ET MF), blast phase PMF (PMF-BP): Consensus on terminology by the international working group for myelofibrosis research and treatment (IWG-MRT)

Cited by 281 publications

References 25 publications

Prodromal myeloproliferative neoplasms: The 2008 WHO classification

Prodromal myeloproliferative neoplasms: The 2008 WHO classification

Efficacy and tolerability of hydroxyurea in the treatment of the hyperproliferative manifestations of myelofibrosis: results in 40 patients

Allo-SCT for Philadelphia-negative myeloproliferative neoplasms in blast phase: a study from the Societe Française de Greffe de Moelle et de Therapie Cellulaire (SFGM-TC)

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