Normal life expectancy for polycythemia vera (PV) patients is possible

Abu‐Zeinah, Ghaith; Silver, Richard T.; Abu-Zeinah, Khalid; Scandura, Joseph M.

doi:10.1038/s41375-021-01447-3

Cited by 22 publications

(23 citation statements)

References 13 publications

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“…This is supported by our previously published findings indicating that the cumulative incidence of myelofibrosis progression is higher than expected in younger compared to older PV patients. 19,20 This is also consistent with the observed higher excess mortality in younger MPN patients compared to older pts. 19 An obvious deficiency of this paper is the fact that documentation of symptoms were found retrospectively on chart review and not prospectively determined.…”

Section: Discussionsupporting

confidence: 86%

Splenomegaly (SPML) in Polycythemia Vera (PV): Its Clinical Significance and its Relation to Symptoms, Post-Polycythemic Myelofibrosis (PPMF), and Survival

et al. 2022

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Section: Discussionsupporting

confidence: 86%

Splenomegaly (SPML) in Polycythemia Vera (PV): Its Clinical Significance and its Relation to Symptoms, Post-Polycythemic Myelofibrosis (PPMF), and Survival

et al. 2022

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“…In this context, MPNs have been described as “A Human Inflammation Model” and “A Human Inflammation Model for Cancer Development”, in which the malignant clone steadily expands in a vicious self-perpetuating cycle fueled by the malignant clone itself [ 14 , 15 ]. Accordingly, early initiation of treatment that directly targets the malignant clone-recombinant interferon-α2 (rIFN-α2) -and the concurrent chronic inflammatory state has been argued to be a prerequisite for a successful outcome of therapeutic intervention [ 22 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 ]. It should be noted that this “Early Interferon Intervention Concept” should preferably be started as early as possible after the MPN diagnosis to prohibit clonal evolution due to inflammation-mediated genomic instability with subclone formation and additional mutations that might confer resistance to treatment, ultimately also mediating myelofibrotic and leukemic transformation.…”

Section: Introductionmentioning

confidence: 99%

“…During the last 30 years, recombinant interferon-α2 has been used in the treatment of MPNs, and its safety and efficacy have been convincingly demonstrated in several studies [ 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 68 , 69 , 70 , 71 , 72 , 73 , 74 , 75 , 76 , 77 , 78 , 79 , 80 , 81 , 82 , 83 , 84 , 85 , 86 , 87 , 88 , 89 , 90 , 91 , 92 , 93 , 94 , 95 , 96 , 97 , 98 , 99 …”

Section: Introductionmentioning

confidence: 99%

Recombinant Interferon-β in the Treatment of Polycythemia Vera and Related Neoplasms: Rationales and Perspectives

Hasselbalch

Skov

Kjær

et al. 2022

Cancers

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About 30 years ago, the first clinical trials of the safety and efficacy of recombinant interferon-α2 (rIFN-α2) were performed. Since then, several single-arm studies have shown rIFN-α2 to be a highly potent anticancer agent against several cancer types. Unfortunately, however, a high toxicity profile in early studies with rIFN-α2 -among other reasons likely due to the high dosages being used-disqualified rIFN-α2, which was accordingly replaced with competitive drugs that might at first glance look more attractive to clinicians. Later, pegylated IFN-α2a (Pegasys) and pegylated IFN-α2b (PegIntron) were introduced, which have since been reported to be better tolerated due to reduced toxicity. Today, treatment with rIFN-α2 is virtually outdated in non-hematological cancers, where other immunotherapies—e.g., immune-checkpoint inhibitors—are routinely used in several cancer types and are being intensively investigated in others, either as monotherapy or in combination with immunomodulatory agents, although only rarely in combination with rIFN-α2. Within the hematological malignancies, rIFN-α2 has been used off-label for decades in patients with Philadelphia-negative chronic myeloproliferative neoplasms (MPNs)—i.e., essential thrombocythemia, polycythemia vera, and myelofibrosis—and in recent years rIFN-α2 has been revived with the marketing of ropeginterferon-α2b (Besremi) for the treatment of polycythemia vera patients. Additionally, rIFN-α2 has been revived for the treatment of chronic myelogenous leukemia in combination with tyrosine kinase inhibitors. Another rIFN formulation-recombinant interferon-β (rIFN-β)—has been used for decades in the treatment of multiple sclerosis but has never been studied as a potential agent to be used in patients with MPNs, although several studies and reviews have repeatedly described rIFN-β as an effective anticancer agent as well. In this paper, we describe the rationales and perspectives for launching studies on the safety and efficacy of rIFN-β in patients with MPNs.

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“…50% can identify patients with polycythemia vera (PV) at higher risk for venous thrombotic events, 10 fewer than a quarter of these patients experienced a venous thrombotic event within 20 years of measurement. Similarly, among 240 patients with available WB MAF in our large single-center PV cohort, 11,12 EFS was indistinguishable by WB MAF for 75% of patients (supplemental Figure 1A, quartiles Q1-Q3). Changes in WB MAF did not predict EFS (supplemental Figure 1B), suggesting that WB MAF was inadequate as a monitoring biomarker.…”

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confidence: 61%

Hematopoietic fitness of JAK2V617F myeloproliferative neoplasms is linked to clinical outcome

et al. 2022

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Classical myeloproliferative neoplasms (MPNs) are chronic, phenotypically diverse malignancies 1 associated with significant morbidity, shortened survival, 2 and limited treatment options. 3 Development of lifeprolonging, potentially curative drugs in MPNs has been more challenging than expected for neoplasms harboring highly recurrent driver mutations that activate targetable tyrosine kinases. Decades of monitoring may be required for analysis of the most important clinical outcomes in MPNs: thrombosis, progression, and mortality. This has prompted use of more convenient clinical trial endpoints with unclear connection to these long-term events. Short-term biomarkers linking MPN biology to event risk may help identify and develop disease-modifying agents with greatest potential to improve event-free survival (EFS). Unfortunately, no such biomarkers are presently available.

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Normal life expectancy for polycythemia vera (PV) patients is possible

Cited by 22 publications

References 13 publications

Splenomegaly (SPML) in Polycythemia Vera (PV): Its Clinical Significance and its Relation to Symptoms, Post-Polycythemic Myelofibrosis (PPMF), and Survival

Splenomegaly (SPML) in Polycythemia Vera (PV): Its Clinical Significance and its Relation to Symptoms, Post-Polycythemic Myelofibrosis (PPMF), and Survival

Recombinant Interferon-β in the Treatment of Polycythemia Vera and Related Neoplasms: Rationales and Perspectives

Hematopoietic fitness of JAK2V617F myeloproliferative neoplasms is linked to clinical outcome

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