Changes in the incidence and overall survival of patients with myeloproliferative neoplasms between 2002 and 2016 in the United States

Verstovšek, Srđan; Yu, Jingbo; Scherber, Robyn M.; Verma, Sumit; Dieyi, Christopher; Chen, Chien Cheng; Parasuraman, Shreekant

doi:10.1080/10428194.2021.1992756

Cited by 37 publications

(14 citation statements)

References 37 publications

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“…The clinical picture is heterogeneous but in general includes progressive cytopenias, organomegaly, debilitating systemic symptoms, and potential for evolution to acute myeloid leukemia. The incidence of MF is 0.44 per 100,000 person-years, with a median age at time of diagnosis of approximately 68 years and a median survival of 5.2–5.9 years [ 4 – 7 ]. Given the highly variable clinical presentation, prognostic scales have been developed to guide treatment decisions.…”

Section: Introductionmentioning

confidence: 99%

Biological drivers of clinical phenotype in myelofibrosis

et al. 2022

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Myelofibrosis (MF) is a myeloproliferative disorder that exhibits considerable biological and clinical heterogeneity. At the two ends of the disease spectrum are the myelodepletive or cytopenic phenotype and the myeloproliferative phenotype. The cytopenic phenotype has a high prevalence in primary MF (PMF) and is characterized by low blood counts. The myeloproliferative phenotype is typically associated with secondary MF (SMF), mild anemia, minimal need for transfusion support, and normal to mild thrombocytopenia. Differences in somatic driver mutations and allelic burden, as well as the acquisition of non-driver mutations further influences these phenotypic differences, prognosis, and response to therapies such as JAK2 inhibitors. The outcome of patients with the cytopenic phenotype are comparatively worse and frequently pose a challenge to treat given the inherent exacerbation of cytopenias. Recent data indicate that an innate immune deregulated state that hinges on the myddosome-IRAK-NFκB axis favors the cytopenic myelofibrosis phenotype and offers opportunity for novel treatment approaches. We will review the biological and clinical features of the MF disease spectrum and associated treatment considerations.

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Section: Introductionmentioning

confidence: 99%

Biological drivers of clinical phenotype in myelofibrosis

et al. 2022

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“…The annual incidence of ET is estimated to be between 1.2 and 3.0 cases per 100,000, 5 , 6 while the estimated prevalence in Western countries is 24-30 cases per 100,000. 7 , 8 The clinical picture is highly variable, with most patients being asymptomatic at presentation.…”

Section: Essential Thrombocythemia In the Realm Of Myeloproliferative...mentioning

confidence: 99%

Aspirin in essential thrombocythemia. For whom? What formulation? What regimen?

Cattaneo¹

2023

haematol

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Essential thrombocythemia (ET) is a BCR-ABL1-negative myeloproliferative neoplasm, the most common clinical manifestations of which include arterial and venous thrombosis, bleeding and vasomotor/microvascular disturbances. Low dose (81-100 mg) aspirin once daily (od), which irreversibly inhibits platelet thromboxane A2 (TxA2) production by acetylating cyclo-oxygenase-1, is the recommended treatment for the control of vascular events in all ET risk categories, except patients at very low risk, who need aspirin for treatment of vasomotor/microvascular disturbances only. Simple observation should be preferred over aspirin prophylaxis in low-risk patients with platelet count >1,000x109/L or harboring CALR mutations. Plain aspirin should be preferred over enteric-coated aspirin because some ET patients display poor responsiveness (“resistance”) to the latter. When treated with od aspirin regimen, adequate inhibition of platelet TxA2 production (measured as serum TxB2) does not persist for 24h in most patients. This phenomenon is associated with the patients’ platelet count and the number (but not the fraction) of circulating immature reticulated platelets with non-acetylated cyclo-oxygenase-1 and is therefore consequent to high platelet production (hallmark of ET), rather than increased platelet turnover (which is normal in ET). Twice daily (bid) aspirin administration overcame this problem and proved safe in small-sized studies. Although additional data on gastrointestinal tolerability will be useful, the bid regimen could already be implemented in clinical practice, considering its favorable risk/benefit profile. However, patients whose platelet count has been normalized could still be treated with the od regimen, because they would otherwise be unnecessarily exposed to a potential small risk of gastrointestinal discomfort.

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“…Mortality rates were higher in patients with BCS and MPNs (RR=3.44, P = 0.05), however, two patients diagnosed with these blood cancers died due to secondary acute myeloid leukemia and extramedullary hematopoiesis in the spleen with consequent organ rupture, events which can occur during disease evolution irrespective of the use of liver transplantation in these cases. Considering 5-year survival rates for PV and ET are of about 80% and of about 50% for PMF, the use of liver transplantation for MPNs-linked BCS is satisfactory in terms of survival prolongation[ 90 ]. Patients with BCS and Philadelphia-negative MPNs receive the same care as those without MPNs during the acute phase.…”

Section: Management Of Budd-chiari Syndrome In Myeloproliferative Neo...mentioning

confidence: 99%

Budd-Chiari syndrome in myeloproliferative neoplasms: A review of literature

Găman¹,

Cozma²,

Manan³

et al. 2023

World J Clin Oncol

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Myeloproliferative neoplasms (MPNs) are defined as clonal disorders of the hematopoietic stem cell in which an exaggerated production of terminally differentiated myeloid cells occurs. Classical, Philadelphia-negative MPNs, i.e. , polycythemia vera, essential thrombocythemia and primary myelofibrosis, exhibit a propensity towards the development of thrombotic complications that can occur in unusual sites, e.g. , portal, splanchnic or hepatic veins, the placenta or cerebral sinuses. The pathogenesis of thrombotic events in MPNs is complex and requires an intricate mechanism involving endothelial injury, stasis, elevated leukocyte adhesion, integrins, neutrophil extracellular traps, somatic mutations ( e.g. , the V617F point mutation in the JAK2 gene), microparticles, circulating endothelial cells, and other factors, to name a few. Herein, we review the available data on Budd-Chiari syndrome in Philadelphia-negative MPNs, with a particular focus on its epidemiology, pathogenesis, histopathology, risk factors, classification, clinical presentation, diagnosis, and management.

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Changes in the incidence and overall survival of patients with myeloproliferative neoplasms between 2002 and 2016 in the United States

Cited by 37 publications

References 37 publications

Biological drivers of clinical phenotype in myelofibrosis

Biological drivers of clinical phenotype in myelofibrosis

Aspirin in essential thrombocythemia. For whom? What formulation? What regimen?

Budd-Chiari syndrome in myeloproliferative neoplasms: A review of literature

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