Thrombosis in essential thrombocytemia and early/prefibrotic primary myelofibrosis: the role of the WHO histological diagnosis

Rupoli, Serena; Goteri, Gaia; Picardi, Paola; Micucci, Giorgia; Canafoglia, Lucia; Scortechini, Anna Rita; Federici, Irene; Giantomassi, Federica; Lio, Lidia Da; Zizzi, Antonio; Honorati, Elisa; Leoni, Pietro

doi:10.1186/s13000-015-0269-1

Cited by 18 publications

(14 citation statements)

References 28 publications

(29 reference statements)

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“…In this context, one should be aware that these data are certainly representing the lower ranges as derived from centers of excellence. The extremely high incidence of 51% pre-PMF cases reported by Rupoli et al 21 was based on the failure to regard only BM specimens with fiber grade 0 (normal) and missing fiber grade 1 (minor) 22,23 as requested by the WHO classification. 1,18 In clinical practice, a rate of pre-PMF mimicking ET ranging between 20 and 30% may be closer to reality.…”

Section: Incidencementioning

confidence: 99%

The new WHO classification for essential thrombocythemia calls for revision of available evidences

et al. 2020

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In the 2016 revised classification of myeloproliferative neoplasms pre-fibrotic primary myelofibrosis (pre-PMF) was recognized as a separate entity, distinct from essential thrombocythemia (ET). Owing that the majority of cases falling in the pre-PMF category were previously diagnosed as ET, one may question about the need to re-evaluate the results of epidemiologic, clinical, and molecular studies, and the results of clinical trials in the two entities. Based on a critical review of recently published studies, pre-PMF usually presents with a distinct clinical and hematological presentation and higher frequency of constitutional symptoms. JAK2V617F and CALR mutations in pre-PMF patients are superimposable to ET, whereas non-driver high-risk mutations are enriched in pre-PMF compared with ET. Thrombosis is not significantly different, whereas bleeding is more frequent in pre-PMF. Median survival is significantly shorter in pre-PMF and 10-year cumulative rates progression to overt myelofibrosis is 0-1% vs. 10-12%, and leukemic transformation is 1-2% vs. 2-6%, in ET and pre-fibrotic-PMF, respectively. Most patients fall in the lower prognostic IPSS group in which observation alone can be recommended. Patients at intermediate risk may require a symptom-driven treatment for anemia, splenomegaly or constitutional symptoms while cytoreductive drugs are indicated in the highrisk category.

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Section: Incidencementioning

confidence: 99%

The new WHO classification for essential thrombocythemia calls for revision of available evidences

et al. 2020

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“…Similarly, patients with prefibrotic PMF had higher 15-year risk of thrombotic events (48 %) compared to WHO-defined ET (17%) or fibrotic PMF patients (16%). By multivariate analysis, age >60 years and prefibrotic PMF were significant risk factors for developing thrombotic complications at 20 years [8]. Barosi et al demonstrated a significantly higher incidence of splanchnic vein thrombosis in prefibrotic PMF as compared to fibrotic PMF [39].…”

Section: Thrombosis In Prefibrotic Pmfmentioning

confidence: 99%

“…It is being increasingly recognized that patients with prefibrotic PMF may have a distinct thrombohemorrhagic risk profile, that might be different from that of either ET or overt PMF patients [11,8]. …”

Section: Introductionmentioning

confidence: 99%

The underappreciated risk of thrombosis and bleeding in patients with myelofibrosis: a review

Falchi

Verstovšek

2017

Ann Hematol

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Bleeding and thrombosis are long recognized complications of myelofibrosis (MF) and contribute significantly to its morbidity and mortality. However, so far, few studies have evaluated the frequency of these events, their characteristics, and their prognostic impact. Based on these studies, thrombotic events in MF are about as common as in essential thrombocytemia (ET) but less common than in polycythemia vera (PV), while bleeding events are relatively more common in MF than in ET or PV. The emergence of the concept of prefibrotic primary MF (PMF), which is associated with a higher frequency of thrombohemorrhagic complications than ET, and the growing evidence that prefibrotic PMF may also have a different thrombotic and bleeding risk profiles than fibrotic (overt) PMF, have emphasized the need for a reappraisal of the risk of thrombosis and hemorrhage in patients with MF. In this review, we discuss the frequency of thrombosis and bleeding in patients with MF, including prefibrotic PMF and their established and potential risk factors.

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“…4 Only a few studies have specifically addressed the issue of thrombosis in patients with PMF. [5][6][7][8] In addition, most often these studies focused on the identification of risk factors for thromboembolism, and the characteristics and distribution of thromboembolic events, and in most cases have not made a distinction between venous thrombosis and arterial thrombosis. To date, no single study performed a direct comparison of thrombosis risk in PMF with concurrent control group without myelofibrosis.…”

Section: Introductionmentioning

confidence: 99%

Association between myelofibrosis and thromboembolism: A population‐based retrospective cohort study

Saliba

Elena

Cohen

et al. 2020

Journal of Thrombosis and Haemostasis

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Background The risk of thromboembolism in myelofibrosis remains incompletely understood. Objectives To examine the association between myelofibrosis and each of venous and arterial thromboembolism. Methods A cohort of 1 469 790 adults without a diagnosis of myelofibrosis was identified on 1 January 2007, from the electronic medical records of the largest health‐care provider in Israel. Participants were followed until 31 December 2016 for the occurrence of myelofibrosis. Four randomly selected controls (without myelofibrosis) were matched to each case of myelofibrosis on age, sex, religious identification, and index date. The two groups were followed from the index date until 31 December 2017 for the occurrence of venous and arterial thromboembolism. Results The study included 642 patients with myelofibrosis and 2568 matched controls. Myelofibrosis was independently associated with increased risk of venous thromboembolism but not with arterial thromboembolism. The propensity score adjusted hazard ratios (HRs) were 6.88 (95% confidence interval [CI], 2.02‐23.45) for venous thromboembolism, and 0.94 (0.49‐1.77) for arterial thromboembolism. Atypical sites of venous thromboembolism occurred almost exclusively in patients with myelofibrosis (four events of Budd Chiari versus none, and two mesenteric vein thrombosis events versus one) and were more likely to occur around the time of myelofibrosis diagnosis. No significant association was found between JAK2 inhibitor treatment (ruxolitinib) and the risk of venous HR 0.97 (0.30‐3.12) or arterial thromboembolism 1.68 (0.78‐3.62). Conclusions Myelofibrosis is associated with increased risk of venous thromboembolism but not of arterial thromboembolism. Atypical sites of venous thromboembolism are more frequent in myelofibrosis and are more likely to occur shortly after diagnosis.

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Thrombosis in essential thrombocytemia and early/prefibrotic primary myelofibrosis: the role of the WHO histological diagnosis

Cited by 18 publications

References 28 publications

The new WHO classification for essential thrombocythemia calls for revision of available evidences

The new WHO classification for essential thrombocythemia calls for revision of available evidences

The underappreciated risk of thrombosis and bleeding in patients with myelofibrosis: a review

Association between myelofibrosis and thromboembolism: A population‐based retrospective cohort study

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