Decrease in JAK2V617F allele burden is not a prerequisite to clinical response in patients with polycythemia vera

Kuriakose, Emil; Vandris, Katherine; Wang, Y. Lynn; Chow, William; Jones, Amy V.; Christos, Paul J.; Cross, Nicholas C. P.; Silver, Richard T.

doi:10.3324/haematol.2011.053348

Cited by 31 publications

(25 citation statements)

References 30 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In regard to the former, results from other studies are divergent 37, 38. In addition, remission was achieved only in patients with baseline palpable splenomegaly ≤5 cm, below the LCM, supporting the findings of other studies 39…”

Section: Discussionsupporting

confidence: 62%

Safety and efficacy of combination therapy of interferon‐α2 and ruxolitinib in polycythemia vera and myelofibrosis

et al. 2018

View full text Add to dashboard Cite

Interferon‐α2 reduces elevated blood cell counts and splenomegaly in patients with myeloproliferative neoplasms (MPN) and may restore polyclonal hematopoiesis. Its use is limited by inflammation‐mediated toxicity, leading to treatment discontinuation in 10‐30% of patients. Ruxolitinib, a potent anti‐inflammatory agent, has demonstrated benefit in myelofibrosis (MF) and polycythemia vera (PV) patients. Combination therapy (CT) with these two agents may be more efficacious than monotherapy with either, potentially improving tolerability of interferon‐α2 as well. We report the preliminary results from a phase II study of CT with pegylated interferon‐α2 and ruxolitinib in 50 MPN patients (PV, n = 32; low‐/intermediate‐1‐risk MF, n = 18), the majority (n = 47) being resistant and/or intolerant to interferon‐α2 monotherapy. Objectives included remission (2013 revised criteria encompassing histologic, hematologic, and clinical responses), complete hematologic response (CHR), molecular response, and toxicity. Follow‐up was 12 months. Partial remission (PR) and sustained CHR were achieved in 9% and 44% of PV patients, respectively. In MF patients, complete or partial remission was achieved in 39%, and sustained CHR in 58%. The median JAK2V617F allele burden declined significantly in both groups. Hematologic toxicity was the most common adverse event and was managed by dose reduction. Thirty‐seven serious adverse events were recorded in 23 patients; the discontinuation rate was 20%. We conclude that CT with interferon‐α2 and ruxolitinib is efficacious in patients with low‐/intermediate‐1‐risk MF and, to a lesser extent, in patients with PV. These preliminary results encourage phase III studies as well as a study with CT in newly diagnosed MPN patients.

show abstract

Section: Discussionsupporting

confidence: 62%

Safety and efficacy of combination therapy of interferon‐α2 and ruxolitinib in polycythemia vera and myelofibrosis

et al. 2018

View full text Add to dashboard Cite

show abstract

“…8 Similar variability in molecular response has been observed with HU. 8 Owing to its reduced use in the JAK2 V617F era, clinical and laboratory data for busulfan are limited. Therefore, we call attention to the effectiveness of busulfan in PV patients refractory to rIFNα and HU, and its efficacy in reducing the JAK2 V617F allele burden in these patients.…”

supporting

confidence: 54%

“…4 The hematologic and molecular responses to recombinant interferon alpha (rIFNα) and hydroxyurea (HU) in PV have been characterized. [5][6][7][8] Sustained molecular responses following pegylated-rIFNα therapy have been observed by some clinicians 5,6 but not by others. 8 Similar variability in molecular response has been observed with HU.…”

mentioning

confidence: 99%

See 1 more Smart Citation

JAK2V617F allele burden is reduced by busulfan therapy: a new observation using an old drug

et al. 2013

Self Cite

View full text Add to dashboard Cite

“…Its pathogenesis is unknown and may be associated with JAK2 mutation (1). The pathological lesions of PV are predominantly involved bone marrow, spleen and liver (2). PV associated with renal disease is clinically rare.…”

Section: Introductionmentioning

confidence: 99%

Polycythemia vera associated with IgA nephropathy: A case report and literature review

Chen¹,

Zhang²,

Li³

et al. 2015

Experimental and Therapeutic Medicine

View full text Add to dashboard Cite

Abstract. A case of polycythemia vera (PV) associated with immunoglobulin A nephropathy (IgAN) in a 57-year-old man is described. The patient had a mild enlargement of the kidneys and elevated serum creatinine level, whereas the glomerular filtration rate was normal. Pathological observation under a light microscope showed mild mesangial hyperplasia. The urinary protein level was found to be positively correlated with changes in blood cell counts. After controlling blood pressure, anti-platelet, hydroxyurea treatment, the patient's proteinuria decreased and renal function remained in the normal range. PV associated with renal disease is rare and generally considered to be associated with hypervolemia or high-viscosity-induced renal hyperperfusion and hyperfiltration. This is a rare case of PV associated with IgAN without a high filtration rate. A review of the clinical features of PV associated with renal disease from previous literature was also conducted. The histological results of the cases varied and included IgAN, focal segmental glomerulosclerosis and membranoproliferative glomerulonephritis.

show abstract

Decrease in JAK2V617F allele burden is not a prerequisite to clinical response in patients with polycythemia vera

Cited by 31 publications

References 30 publications

Safety and efficacy of combination therapy of interferon‐α2 and ruxolitinib in polycythemia vera and myelofibrosis

Safety and efficacy of combination therapy of interferon‐α2 and ruxolitinib in polycythemia vera and myelofibrosis

JAK2V617F allele burden is reduced by busulfan therapy: a new observation using an old drug

Polycythemia vera associated with IgA nephropathy: A case report and literature review

Contact Info

Product

Resources

About