Diagnostic impact of the 2016 revised who criteria for polycythemia vera

Barbui, Tiziano; Thiele, Jüergen; Gisslinger, Heinz; Carobbio, Alessandra; Vannucchi, Alessandro M.; Tefferi, Ayalew

doi:10.1002/ajh.24684

Cited by 28 publications

(43 citation statements)

References 10 publications

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“…PV patients with early stage disease represent a group which were not “captured” by the formerly used cutoffs for Hb and/or Hct . A proportion of these patients may be wrongly diagnosed as ET or MPN‐U.…”

Section: Discussionmentioning

confidence: 99%

“…In addition, the diagnostic accuracy of different Hb or Hct threshold values as surrogate markers for RCM have been considered but no firm conclusion has ever been reached . However, recent studies, irrespectively of the chosen approach, have shown that a meaningful discrimination between PV and ET may not be achieved without an adequate BM evaluation, an important component of diagnostic algorithms for PV and ET diagnoses in the 2016 revised WHO classification …”

Section: Discussionmentioning

confidence: 99%

“…In fact, using the BCSH criteria 6,7 the fraction of mPV patients decreased from 35% to 15%. 13,14 Following proposals of experts, 15 a Hb level of 16.5 g/dL in men and 16.0 g/dL for women or a Hct level of 49% in men and 48% in women was determined to be the optimal cutoff levels for distinguishing JAK2mutated ET from mPV 13 and recently adopted by the 2016 revised WHO classification. 5 In this regard, correct diagnosis of mPV constitutes an important issue and will certainly improve clinical management of patients while avoiding undertreatment and prevention of thrombotic events.…”

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confidence: 99%

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European LeukemiaNet study on the reproducibility of bone marrow features in masked polycythemia vera and differentiation from essential thrombocythemia

et al. 2017

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The purpose of the study was to assess consensus and interobserver agreement among an international panel of six hematopathologists regarding characterization and reproducibility of bone marrow (BM) histologic features used to diagnose early stage myeloproliferative neoplasms, in particular differentiation of so-called masked/prodromal polycythemia vera (mPV) from JAK2-mutated essential thrombocythemia (ET). The six members of the hematopathology panel evaluated 98 BM specimens independently and in a blinded fashion without knowledge of clinical data. The specimens included 48 cases of mPV according to the originally published hemoglobin threshold values for this entity (male: 16.0-18.4 g/dL, female: 15.0-16.4 g/dL), 31 cases with overt PV according to the updated 2016 WHO criteria, and 19 control cases. The latter group included cases of JAK2-mutated ET, primary myelofibrosis, myelodysplastic syndrome, and various reactive conditions. Inter-rater agreement between the panelists was very high (overall agreement 92.6%, kappa 0.812), particularly with respect to separating mPV from ET. Virtually all cases of mPV were correctly classified as PV according to their BM morphology. In conclusion, a central blinded review of histology slides by six hematopathologists demonstrated that highly reproducible specific histological pattern characterize PV and confirmed the notion that there are no significant differences between mPV and overt PV in relation to BM morphology.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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European LeukemiaNet study on the reproducibility of bone marrow features in masked polycythemia vera and differentiation from essential thrombocythemia

et al. 2017

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“…The latter point is equally important when interpreting results and conclusions from historical as well as more contemporary natural history studies in “ET”. Fortunately, the particular concept has been endorsed by the WHO subcommittee on MPN and applied in a number of relevant papers on the subject matter …”

Section: Discussionmentioning

confidence: 99%

Gender and survival in essential thrombocythemia: A two‐center study of 1,494 patients

et al. 2017

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Based on suggestive information from recent epidemiologic data and earlier retrospective studies, we revisited the effect of gender on survival in 1,494 patients with essential thrombocythemia (ET). The primary study population included 904 patients from the Mayo Clinic (median age 58 years; 65% females); risk distribution, according to the international prognostic score for ET (IPSET), was 23% high, 42% intermediate and 35% low. Multivariable analysis that included IPSET-relevant risk factors identified male sex (HR 1.6, 95% CI 1.3-2.0), age ≥60 years (HR 4.3, 95% CI 3.4-5.4) and leukocyte count ≥11 × 10(9)/L (HR 1.5, 95% CI 1.3-1.9) as independent predictors of shortened survival. These findings were confirmed by analysis of a separate cohort of 590 ET patients (65% females) from the University of Florence, Italy, with corresponding HRs (95% CI) of 1.6 (1.1-2.5), 4.6 (2.2-9.5) and 1.8 (1.1-2.8). The independent prognostic effect of gender was further corroborated by a separate multivariable analysis against IPSET risk categories; HR (95% CI) for the Mayo Clinic/Florence cohorts were 1.5/1.6 (1.2/1.1-1.8/2.5) for male sex, 6.8/7.5 (5.0/3.1-9.3/18.3) for IPSET high risk and 2.8/4.1 (2.1/1.8-3.8/9.5) for IPSET intermediate risk. Furthermore, the survival disadvantage in men was most apparent in IPSET high risk category and in patients older than 60 years. In both patient cohorts, thrombosis history garnered significance in univariate, but not in multivariable analysis. The observations from the current study suggest that women with ET live longer than their male counterparts and that gender might supersede thrombosis history as a risk variable for overall survival.

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“…Within the WHO MPN category, polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) are operationally grouped together as “the JAK2 MPNs” . PMF is further sub‐classified into “prefibrotic” and “overtly fibrotic” PMF .…”

Section: Disease Overviewmentioning

confidence: 99%

Primary myelofibrosis: 2019 update on diagnosis, risk‐stratification and management

2018

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Observation alone is advised for MIPSS70+ version 2.0 "low" and "very low" risk disease (estimated 10-year survival 56%-92%); allogeneic stem cell transplant is the preferred treatment of choice for "very high" and "high" risk disease (estimated 10-year survival 0-13%); treatment-requiring patients with intermediate-risk disease (estimated 10-year survival 30%) are best served by participating in clinical trials. All other treatment approaches, including the use of JAK2 inhibitors, are mostly palliative and should not be used in the absence of clear treatment indications. Conventional treatment for anemia includes androgens, prednisone, thalidomide and danazol, for symptomatic splenomegaly hydroxyurea and ruxolitinib and for constitutional symptoms ruxolitinib. Splenectomy is considered for drug-refractory splenomegaly and involved field radiotherapy for nonhepatosplenic EMH and extremity bone pain.

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Diagnostic impact of the 2016 revised who criteria for polycythemia vera

Cited by 28 publications

References 10 publications

European LeukemiaNet study on the reproducibility of bone marrow features in masked polycythemia vera and differentiation from essential thrombocythemia

European LeukemiaNet study on the reproducibility of bone marrow features in masked polycythemia vera and differentiation from essential thrombocythemia

Gender and survival in essential thrombocythemia: A two‐center study of 1,494 patients

Primary myelofibrosis: 2019 update on diagnosis, risk‐stratification and management

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