Pegylated interferon alpha 2a is an effective and well‐tolerated treatment option for lymphocyte‐variant hypereosinophilic syndrome

Choi, Charles Q.; Møller, Daniél Vega; Tan, Jessie; Dou, Carol; Peterson, Erica A.; Medvedev, N N; Dutz, Jan P.; Carruthers, Mollie; Chen, Luke Y C

doi:10.1111/bjh.16332

Cited by 15 publications

(22 citation statements)

References 20 publications

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“…Two patients in our series presented a complete response to a weekly dose of 180 μ with good clinical tolerance, but one had to stop treatment due to severe neutropenia. Another group has recently reported prolonged steroid-free remission in response to peg-IFN-α in 4/5 patients with CD3 − CD4 + T cell associated L-HES ranging from 10 to 95 months, with significant reductions in abnormal T cell counts ( 32 ). However, the fifth patient described in more detail as a case report ( 33 ) experienced disease recurrence while on therapy within months, as observed in two patients in our series with recombinant IFN-α.…”

Section: Discussionmentioning

confidence: 99%

Eosinophilia Associated With CD3−CD4+ T Cells: Characterization and Outcome of a Single-Center Cohort of 26 Patients

et al. 2020

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Section: Discussionmentioning

confidence: 99%

Eosinophilia Associated With CD3−CD4+ T Cells: Characterization and Outcome of a Single-Center Cohort of 26 Patients

et al. 2020

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“…These studies demonstrated overall excellent efficacy, with complete or partial responses in all patients. 18 By contrast, Carpentier et al 15 demonstrate somewhat reduced efficacy, with five out of 10 patients who received the medication demonstrating at least an initial response. It should be noted that this study used more IFNa than peg-IFNa, with various dosing regimens, perhaps explaining some of the differences in response.…”

Section: Interferon-alpha (Ifn-a)mentioning

confidence: 96%

“…In our centre, patients are generally considered for steroid-sparing therapies when the patient cannot taper below 10 mg of prednisone or equivalents/day. 18 Any patient requiring >10 mg prednisone equivalents/day in Carpentier et al 15 was also given second-line, steroid-sparing therapy. Overt treatment failure of corticosteroids was much less common, with very few patients (nine of 102) not responding at least partially to 1 mg/kg/day.…”

Section: Corticosteroidsmentioning

confidence: 99%

“…21,22 In the published literature, 40 patients were treated with IFN-a as either IFNa-2b, with doses ranging from 7 to 42 million iu/week divided into daily doses, or peg-IFNa (most commonly peg-IFNa-2a) with doses ranging from 45 µg weekly or 90 µg every 2 weeks to 180 µg once weekly. 15,17,18 Overall, 32 of the 40 patients with L-HES receiving IFNa had a complete or partial response, which permitted significant reduction or discontinuation of steroids in most cases (complete data available from the authors on request). 3,18 The earliest studies documenting the use of IFNa for L-HES used the non-pegylated form.…”

Section: Interferon-alpha (Ifn-a)mentioning

confidence: 99%

“…15,17,18 Overall, 32 of the 40 patients with L-HES receiving IFNa had a complete or partial response, which permitted significant reduction or discontinuation of steroids in most cases (complete data available from the authors on request). 3,18 The earliest studies documenting the use of IFNa for L-HES used the non-pegylated form. The first report, in 1994, detailed successful treatment with IFNa-2b at a dose of 5 million iu/day.…”

Section: Interferon-alpha (Ifn-a)mentioning

confidence: 99%

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Treatment of lymphocyte‐variant hypereosinophilic syndrome (L‐HES): what to consider after confirming the elusive diagnosis

2021

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Lymphocyte-variant hypereosinophilic syndrome (L-HES) is a rare disease driven by immunophenotypically aberrant T cells producing eosinophilopoetic cytokines such as interleukin-5 (IL-5). Treatment is challenging because L-HES is relatively steroid resistant and not amenable to tyrosine kinase inhibitors. We searched the literature for clinical trials and observational studies, including case reports, of patients treated for L-HES. In all, 25 studies were selected; two were randomised controlled trials of IL-5 blockade, which included some patients with L-HES, and the rest were observational studies. Corticosteroids are often used as first-line therapy, but patients with L-HES have lower response rates than other types of HES. Treatments that reduce symptoms and steroid dependence in some patients include interferonalpha (IFN-a), anti-IL-5 monoclonal antibodies, cyclosporine and mycophenolate. These drugs target T-cell activation and proliferation, or IL-5 directly. Although effective, IFN-a and cyclosporine were commonly reported to cause side-effects resulting in discontinuation. Alemtuzumab can induce remissions, but these are generally short lived. The anti-IL-5 monoclonal antibodies mepolizumab and benralizumab are effective and well tolerated, but with a high rate of relapse once withdrawn. Hydroxyurea, methotrexate, imatinib were unsuccessful in most patients studied. More prospective clinical trials are needed for patients with L-HES.

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World Health Organization‐defined eosinophilic disorders: 2022 update on diagnosis, risk stratification, and management

2021

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Disease Overview: The eosinophilias encompass a broad range of nonhematologic (secondary or reactive) and hematologic (primary or clonal) disorders with potential for end-organ damage.Diagnosis: Hypereosinophilia (HE) has generally been defined as a peripheral blood eosinophil count greater than 1.5 Â 10 9 /L. After exclusion of secondary causes of eosinophilia, diagnostic evaluation of primary eosinophilias relies on morphologic review of the blood and marrow, standard cytogenetics, fluorescence in situ hybridization, next generation sequencing gene assays, and flow immunophenotyping to detect histopathologic or clonal evidence for an acute or chronic hematolymphoid neoplasm.Risk Stratification: Disease prognosis relies on identifying the subtype of eosinophilia. After evaluation of secondary causes of eosinophilia, the 2016 World Health Organization endorses a semi-molecular classification scheme of disease subtypes. This includes the major category "myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRA, PDGFRB, or FGFR1 or with PCM1-JAK2", and the myeloproliferative neoplasm subtype, "chronic eosinophilic leukemia, not otherwise specified" (CEL, NOS). Lymphocyte-variant HE is an aberrant T-cell clone-driven reactive eosinophila, and idiopathic hypereosinophilic syndrome (HES) is a diagnosis of exclusion. Risk-adapted Therapy:The goal of therapy is to mitigate eosinophil-mediated organ damage. For patients with milder forms of eosinophilia (eg, < 1.5 Â 10 9 /L) without symptoms or signs of organ involvement, a watch and wait approach with close follow-up may be undertaken. Identification of rearranged PDGFRA or PDGFRB is critical because of the exquisite responsiveness of these diseases to imatinib. Corticosteroids are first-line therapy for patients with lymphocyte-variant HE and HES.Hydroxyurea and interferon-α have demonstrated efficacy as initial treatment and in steroid-refractory cases of HES. Mepolizumab, an interleukin-5 (IL-5) antagonist monoclonal antibody, was recently approved by the US Food and Drug Administration for patients with idiopathic HES. The use of the IL-5 receptor antibody benralizumab, as well as other targeted therapies such as JAK2 and FGFR1 inhibitors, is under active investigation.

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Pegylated interferon alpha 2a is an effective and well‐tolerated treatment option for lymphocyte‐variant hypereosinophilic syndrome

Cited by 15 publications

References 20 publications

Eosinophilia Associated With CD3−CD4+ T Cells: Characterization and Outcome of a Single-Center Cohort of 26 Patients

Eosinophilia Associated With CD3−CD4+ T Cells: Characterization and Outcome of a Single-Center Cohort of 26 Patients

Treatment of lymphocyte‐variant hypereosinophilic syndrome (L‐HES): what to consider after confirming the elusive diagnosis

World Health Organization‐defined eosinophilic disorders: 2022 update on diagnosis, risk stratification, and management

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