Epidemiology of myeloproliferative neoplasms in the United States

Abstract

Myelofibrosis (MF), polycythemia vera (PV) and essential thrombocythemia (ET) are three classic BCR ABL fusion gene-negative chronic myeloproliferative neoplasms (MPNs). Though rare, it is important to understand the burden of illness of these disorders for public health planning, healthcare insurers and pharmaceutical manufacturers. Therefore, we have described the incidence of MF and prevalence of MF, ET and PV in the United States between 2008 and 2010 based on data from two large health plans. The incidence of primary MF was about 1 per 100 000 per year and did not vary over the study years. The prevalence of PV (44-57 per 100 000) and ET (38-57 per 100 000) was much higher than that of MF (4-6 per 100 000) or subgroups containing MF (post-PV MF = 0.3-0.7 per 100 000; post-ET MF = 0.5-1.1 per 100 000). Additional research using other national databases and/or study designs is needed to substantiate these findings.

Understanding deregulated cellular and molecular dynamics in the haematopoietic stem cell niche to develop novel therapeutics for bone marrow fibrosis

“…The majority of patients with PMF carry mutations that activate JAK–STAT signalling; 60% harbour the JAK2V617F mutation, approximately 30% carry a calreticulin (CALR) mutation, and 8% carry a myeloproliferative leukaemia virus oncogene ( MPL ) mutation 3, 4, 5, 6, 7, 8. PMF is the most aggressive of the three classic MPNs, and is associated with significantly shortened survival 9, 10. Despite our advanced understanding of the molecular pathogenesis of malignant myeloid diseases, the stepwise pathogenesis of BM fibrosis is poorly understood.…”

Identification of JAK2V617F Mutation on Myeloproliferative Disorders in Medan

“…The incidence of PMF in 2008 to 2010 was estimated to be 1 per 100,000 population. The median age at diagnosis is 67 years [5]. As noted earlier, patients with PV and other MPNs can develop myelofibrosis late in their disease course JAK2 gene provides instructions for making a protein that promotes the growth and division (proliferation) of cells.…”

“…In the United States, the estimated prevalence of PV ranges from 44 to 57 cases per 100,000 people [5]. PV typically occurs in the 6th or 7th decade of life and occurs more commonly in men and in both men and women of East EuropeanJewish ancestry [6,7].…”

Safety of ruxolitinib therapy prior to allogeneic hematopoietic stem-cell transplantation for myeloproliferative neoplasms

“…1,2 It has a mean survival of 6.5 years from the time of diagnosis but significant heterogeneity is present, and the survival is much shorter for patients with intermediate and high-risk disease. 3 Despite the rapid increase in the number of novel therapeutics being tested for the treatment of myelofibrosis, allogeneic hematopoietic cell transplant (AHCT) remains the only curative therapy.…”