Low-risk polycythemia vera and essential thrombocythemia: management considerations and future directions

Goulart, Hannah; Mascarenhas, John; Tremblay, Douglas

doi:10.1007/s00277-022-04826-7

Cited by 6 publications

(3 citation statements)

References 88 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…For these reasons, there is growing interest in the active treatment of low-risk patients with PV and ET [ 35 , 36 ]. The European Leukemia Net (ELN) 2021 recommendations suggest that low-risk patients with PV should begin cytoreductive drug therapy if at least one of the following criteria are fulfilled: (1) strictly defined intolerance to phlebotomy, (2) symptomatic progressive splenomegaly, (3) persistent leukocytosis, (4) progressive leukocytosis, (5) extreme thrombocytosis, (6) inadequate hematocrit control requiring phlebotomy, (7) persistently high cardiovascular risk, and (8) persistently high symptom burden.…”

Section: Unmet Needs In Current Pv/et Treatmentmentioning

confidence: 99%

Novel therapeutic strategies for essential thrombocythemia/polycythemia vera

Yoon

Won

2023

Blood Res

View full text Add to dashboard Cite

Myeloproliferative neoplasms (MPNs) are clonal disorders of hematopoietic stem cells; these include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). MPNs are inflammatory cancers, wherein the malignant clone generates cytokines that sustain the inflammatory drive in a self-perpetuating vicious cycle. The course of MPNs follows a biological continuum, that is, from early cancer stages (ET/PV) to advanced myelofibrosis as well as impending leukemic transformation. MPN-related symptoms, e.g., fatigue, general weakness, and itching, are caused by inflammatory cytokines. Thrombosis and bleeding are also exacerbated by inflammatory cytokines in patients with MPN. Until recently, the primary objective of ET and PV therapy was to increase survival rates by preventing thrombosis. However, several medications have recently demonstrated the ability to modify the course of the disease; symptom relief is expected for most patients. In addition, there is increasing interest in the active treatment of patients at low risk with PV and ET. This review focuses on the ET/PV treatment strategies as well as novel treatment options for clinical development.

show abstract

Section: Unmet Needs In Current Pv/et Treatmentmentioning

confidence: 99%

Novel therapeutic strategies for essential thrombocythemia/polycythemia vera

Yoon

Won

2023

Blood Res

View full text Add to dashboard Cite

show abstract

“…However, when comparing the mortality rates of healthy individuals and patients with MPN in the same age group, www.kjim.org https://doi.org/10.3904/kjim.2023.333 the relative risk in the younger group was greater than in the older group [49]. Thus, the treatment of young patients necessitates considerable care [50,51]. Long-acting IFNα, which is effective at regulating blood cell counts and alleviating symptoms, has been shown to have the potential to modify the disease by decreasing JAK2 V617F VAF and is anticipated to play a significant role.…”

Section: Future Direction Of Long-acting Ifnα In Patients With Mpnmentioning

confidence: 99%

Long-acting interferon: pioneering disease modification of myeloproliferative neoplasms

Yoon,

Kim

2023

Korean J Intern Med

View full text Add to dashboard Cite

Myeloproliferative neoplasms (MPNs) are clonal disorders of hematopoietic stem cells. The malignant clones produce cytokines that drive self-perpetuating inflammatory responses and tend to transform into more aggressive clones, leading to disease progression. The progression of MPNs follows a biological sequence from the early phases of malignancy, polycythemia vera, and essential thrombocythemia, to advanced myelofibrosis and leukemic transformation. To date, the treatment of MPNs has focused on preventing thrombosis by decreasing blood cell counts and relieving disease-related symptoms. However, interferon (IFN) has been used to treat MPNs because of its ability to attack cancer cells directly and modulate the immune system. IFN also has the potential to modulate diseases by inhibiting JAK2 mutations, and recent studies have demonstrated clinical and molecular improvements. Long-acting IFN is administered less frequently and has fewer adverse effects than conventional IFN. The current state of research on long-acting IFN in patients with MPNs is discussed, along with future directions.

show abstract

“…Current information on risk factors of the major critical events (thrombosis, evolution to MF, blast phase (BP), and survival) derives from registry and multicenter observational studies while single-center reports conducted at tertiary referral institutions are very limited [ 5 ]. Each study design has its strengths and limitations.…”

mentioning

confidence: 99%