An accurate, simple prognostic model consisting of age,
 JAK2
 ,
 CALR
 , and
 MPL
 mutation status for patients with primary myelofibrosis

Rozovski, Uri; Verstovšek, Srđan; Manshouri, Taghi; Dembitz, Vilma; Božinović, Ksenija; Newberry, Kate J.; Zhang, Ying; Bove, Joseph E.; Pierce, Sherry; Kantarjian, Hagop M.; Estrov, Zeev

doi:10.3324/haematol.2016.149765

Cited by 34 publications

(29 citation statements)

References 32 publications

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“…To study the association between PTX3, CRP and SAP plasma levels and PMF patient outcome, pentraxin plasma levels were dichotomized into high and low levels using the maximum χ 2 value of the Mantel–Cox test, as previously described (Rozovski et al , ). According to this algorithm, high PTX3 levels (≥7·0 ng/ml) or high CRP levels (≥6·6 mg/l) were associated with an unfavourable overall survival, as estimated by using the Kaplan–Meier method (Fig B, left and middle panels, respectively; log‐rank test: P = 0·044 and P = 0·01, respectively).…”

Section: Multivariate‐adjusted Risk Of Death Associated With High Penmentioning

confidence: 99%

Pentraxin‐3 plasma levels correlate with tumour burden and overall survival in patients with primary myelofibrosis

et al. 2018

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Section: Multivariate‐adjusted Risk Of Death Associated With High Penmentioning

confidence: 99%

Pentraxin‐3 plasma levels correlate with tumour burden and overall survival in patients with primary myelofibrosis

et al. 2018

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“…A first attempt to define prognosis of PMF patients in an easier way was made by Rozovski et al: In details, they proposed a prognostic model that includes only age, JAK2 V617F allele burden, and triple‐negative mutational status and subdivides patients in a low‐ and a high‐risk category. However, they did not consider at all morphological features of their patients, including GBMF …”

Section: Introductionmentioning

confidence: 99%

“…However, they did not consider at all morphological features of their patients, including GBMF. 30 The present multicenter study developed instead a simple method to integrate, at diagnosis, the prognostic information from two welldefined prognostic scoring systems (IPSS and GBMF) with the molecular status (MS) of PMF patients to obtain an overall prognostic stratification of patients applicable worldwide, even in the absence of data on "other" mutations.…”

Section: Introductionmentioning

confidence: 99%

Integrating clinical, morphological, and molecular data to assess prognosis in patients with primary myelofibrosis at diagnosis: A practical approach

Iurlo

Elli

Palandri

et al. 2019

Hematological Oncology

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Currently available prognostic scoring systems in primary myelofibrosis (PMF) do not integrate clinical, histological, and molecular data, or they also required information on “other” mutations that are available in the clinical practice only in a very limited number of laboratories. In the present multicenter study, including 401 PMF patients, an integrated International Prognostic Scoring System (I‐IPSS) was developed by combining IPSS, grade of bone marrow fibrosis (GBMF), and driver mutations molecular status (MS) to define PMF prognosis at diagnosis. Four prognostic categories were identified: I‐IPSS–low risk (113 patients), I‐IPSS–intermediate‐1 risk (56 patients), I‐IPSS–intermediate‐2 risk (154 patients), and I‐IPSS–high risk (78 patients). Median overall survival was 26.7 years in I‐IPSS–intermediate‐1, 10.8 in I‐IPSS–intermediate‐2, and 6.4 in I‐IPSS‐high‐risk patients (log‐rank test <0.0001); instead, it was not reached in the I‐IPSS–low‐risk cohort because of the extremely low number of registered deaths. The addition of GBMF and MS to IPSS improved the efficacy for predicting the risk of death. Indeed, the sensitivity of I‐IPSS was significantly higher (P < .05) than that of IPSS, considering both total deaths and 5‐ and 10‐year mortality. This comprehensive approach allows clinicians to evaluate mutual interactions between IPSS, GBMF, and MS and identify high‐risk patients with poor prognosis who may benefit from aggressive treatments. More importantly, this integrated score can be easily applicable worldwide as it only required information that represent the good clinical practice in the management of PMF patients.

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“…Finally, with the growing database of the mutational landscape in patients with MPN, efforts are underway to incorporate mutational patterns into the prognostic classification of these disorders. Mutations in three genes, JAK2, CALR , and MPL , have been identified as “driver mutations”, and have been included, in addition to patient age (and along with other high‐risk mutations), in an initial model that considers the impact of mutational status on prognosis (mutation enhanced IPSS) in the form of the MIPSS70 and the MIPSS70 Plus (Table ) . Most recently, further classifications have been proposed, focusing on high‐risk karyotype and additional mutations .…”

Section: Introductionmentioning

confidence: 99%

Genetics, prognosis, and transplantation for myelofibrosis

et al. 2018

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Primary myelofibrosis (PMF), polycythemia vera (PV), and essential thrombocythemia (ET) are chronic disorders that may extend over years or decades. Therapy tends to be conservative, but once marrow fibrosis and peripheral cytopenias become the dominant characteristics, prognosis is poor. Hematopoietic cell transplantation (HCT) is the only treatment with curative potential, leading to survival in remission in 35%‐70% of patients. However, HCT is associated with risks, and despite the development of numerous risk scoring systems, optimal timing of HCT remains controversial. The identification of “driver mutations” in JAK2, MPL1, and CALR, and prognostically relevant additional mutations, may assist in the decision‐making process. While patients with type 1 CALR mutations generally have a superior prognosis, absence of all driver mutations is associated with inferior outcome. Mutations in ASXL1, SRSF2, IDH1/2, and EZH2 are linked to more rapid disease progression and, along with biallelic TP53 mutations, leukemic transformation. The strongest risk factor is the presence of multiple mutations. By MIPSS70 criteria, considering mutations, median survival was 27 years for the best risk group, but 2.3 years for the highest‐risk group. The presence of nondriver mutations, particularly in the absence of CALR mutations, and association with adverse cytogenetics, should lead to consideration of HCT. But the role of mutations has to be assessed in the context of the overall presentation. Unfortunately, risk factors that affect the natural history of the disease also impact post‐HCT outcome. Needed are innovative transplant strategies, also including pre‐HCT and post‐HCT adjuvant therapy.

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An accurate, simple prognostic model consisting of age, JAK2 , CALR , and MPL mutation status for patients with primary myelofibrosis

Cited by 34 publications

References 32 publications

Pentraxin‐3 plasma levels correlate with tumour burden and overall survival in patients with primary myelofibrosis

Pentraxin‐3 plasma levels correlate with tumour burden and overall survival in patients with primary myelofibrosis

Integrating clinical, morphological, and molecular data to assess prognosis in patients with primary myelofibrosis at diagnosis: A practical approach

Genetics, prognosis, and transplantation for myelofibrosis

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