The clinical role of interferon alpha in Philadelphia-negative myeloproliferative neoplasms

Yoon, Seug Yun; Won, Jong-Ho

doi:10.5045/br.2021.2020334

Cited by 9 publications

(7 citation statements)

References 51 publications

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“…Since the introduction of pegylated (PEG)-IFNa, which reduces both dosing intervals and side-effects compared with the non-pegylated form, IFNa has regained interest as a potential tool to interfere with the disease course, via potently inducing hematologic and molecular responses in a majority of ET and PV patients [ 87 ]. In MF patients, the overall response rate (ORR) is lower than in ET or PV patients, and complete responses are rare [ 89 ]. In this subtype, combination treatments with ruxolitinib may achieve better results [ 90 , 91 ].…”

Section: Novel Therapeutics Targeting Mpn Progressionmentioning

confidence: 99%

Progression of Myeloproliferative Neoplasms (MPN): Diagnostic and Therapeutic Perspectives

Baumeister

Chatain

Sofias

et al. 2021

Cells

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Classical BCR-ABL-negative myeloproliferative neoplasms (MPN) are a heterogeneous group of hematologic malignancies, including essential thrombocythemia (ET), polycythemia vera (PV), and primary myelofibrosis (PMF), as well as post-PV-MF and post-ET-MF. Progression to more symptomatic disease, such as overt MF or acute leukemia, represents one of the major causes of morbidity and mortality. There are clinically evident but also subclinical types of MPN progression. Clinically evident progression includes evolution from ET to PV, ET to post-ET-MF, PV to post-PV-MF, or pre-PMF to overt PMF, and transformation of any of these subtypes to myelodysplastic neoplasms or acute leukemia. Thrombosis, major hemorrhage, severe infections, or increasing symptom burden (e.g., pruritus, night sweats) may herald progression. Subclinical types of progression may include increases in the extent of bone marrow fibrosis, increases of driver gene mutational allele burden, and clonal evolution. The underlying causes of MPN progression are diverse and can be attributed to genetic alterations and chronic inflammation. Particularly, bystander mutations in genes encoding epigenetic regulators or splicing factors were associated with progression. Finally, comorbidities such as systemic inflammation, cardiovascular diseases, and organ fibrosis may augment the risk of progression. The aim of this review was to discuss types and mechanisms of MPN progression and how their knowledge might improve risk stratification and therapeutic intervention. In view of these aspects, we discuss the potential benefits of early diagnosis using molecular and functional imaging and exploitable therapeutic strategies that may prevent progression, but also highlight current challenges and methodological pitfalls.

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Section: Novel Therapeutics Targeting Mpn Progressionmentioning

confidence: 99%

Progression of Myeloproliferative Neoplasms (MPN): Diagnostic and Therapeutic Perspectives

Baumeister

Chatain

Sofias

et al. 2021

Cells

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“…Efficacy evaluation criteria were referred to the prior literature [ 9 ]. Progression-free survival (PFS) refers to the duration from the date of enrollment to the first occurrence of disease progression (new thrombosis, bleeding events, progressive enlargement of the spleen, myelodysplastic syndrome (MDS), myelofibrosis (MF), aggravation of the original myelofibrosis reticulum staining, or acute myeloid leukemia (AML) caused by peripheral cytopenia), or from the date of enrollment to the time of death from any cause.…”

Section: Methodsmentioning

confidence: 99%

Analysis of the Clinical Significance and Safety of Interferon in the Treatment of Chronic Myeloproliferative Tumors

Yin

et al. 2022

Journal of Oncology

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Objective. To investigate the clinical significance and safety of interferon in the treatment of chronic myeloproliferative tumors (MPN). Methods. In this prospective study, a total of 120 patients with advanced chronic MPN admitted to our hospital between April 2016 and August 2020 were assessed for eligibility and recruited, including 62 patients with JAK2V617F mutation-positive ET (ET group) and 58 patients with JAK2V617F mutation-positive PV (PV group). 62 patients with JAK2V617F mutation-positive ET were assigned (1 : 1) to receive interferon-α (IFN-α) or hydroxyurea (HU). A similar subgrouping method for treatment of IFN-α and HU was introduced to patients with JAK2V617F mutation-positive PV. Outcome measures included efficacy and adverse reactions. Results. For patients with JAK2V617F mutation-positive ET and PV, there were no significant differences in the overall response rate between the groups treated with IFN-α or HU ( P > 0.05 ); however, the patients treated with IFN-α had a significantly higher 5-year progression-free survival (PFS) than those treated with HU ( P < 0.05 ). IFN-α was associated with a significantly lower incidence of disease progression, thrombotic events, splenomegaly, myelofibrosis, nausea, and vomiting and a higher incidence of hematological adverse reactions and flu-like symptoms versus HU ( P < 0.05 ). After six months of treatment, the PV group had 12 cases of hematological response both in the IFN-α subgroup and the HU subgroup and fewer PV patients treated with IFN-α required phlebotomy versus those treated with HU ( P < 0.05 ), in which 4 patients in the IFN-α subgroup had no hematological response and 6 patients in the HU subgroup had no hematological response. There was no significant difference in the number of cases with phlebotomy between the two subgroups of PV patients without hematological response ( P > 0.05 ). Conclusion. The use of IFN in the treatment of JAK2V617F mutation-positive ET and PV patients yields a prominent clinical effect by prolonging PFS and avoiding phlebotomy for JAK2V617F mutation-positive PV patients.

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“…To capture these symptoms directly from patients with MPNs, the Myelofibrosis Symptom Assessment Form was developed, which was subsequently modified to include ET and PV to form the Myeloproliferative Neoplasm Symptom Assessment Form (MPN-SAF) [ 24 , 25 ]. Numerous studies have shown that ruxolitinib and interferon (IFN) are effective in the symptomatic management of patients with MPNs [ 26 , 27 ]. Complete responses were obtained in patients with PV and ET who were treated with either hydroxyurea (HU) or pegylated interferon alfa-2a (peg IFNα-2a); however, only 19–32% of the patients reported clinically significant improvement in symptom reduction [ 28 ].…”

Section: Unmet Needs In Current Pv/et Treatmentmentioning

confidence: 99%

“…With the development of peg IFNα-2a and ropeg IFNα-2b, side effects have been reduced and administration intervals have been extended. Therefore, IFN is a promising treatment of choice for disease modification, especially given its impact on mutation burden [ 27 ].…”

Section: Novel Agents For Pv/et Treatmentmentioning

confidence: 99%

Novel therapeutic strategies for essential thrombocythemia/polycythemia vera

Yoon

Won

2023

Blood Res

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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.

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The clinical role of interferon alpha in Philadelphia-negative myeloproliferative neoplasms

Cited by 9 publications

References 51 publications

Progression of Myeloproliferative Neoplasms (MPN): Diagnostic and Therapeutic Perspectives

Progression of Myeloproliferative Neoplasms (MPN): Diagnostic and Therapeutic Perspectives

Analysis of the Clinical Significance and Safety of Interferon in the Treatment of Chronic Myeloproliferative Tumors

Novel therapeutic strategies for essential thrombocythemia/polycythemia vera

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