Romidepsin is an effective and well-tolerated therapy in CD3−CD4+ lymphocyte-variant hypereosinophilic syndrome: A case report

Baulier, G.; Asli, Bouchra; Galicier, Lionel; Fieschi, Claire; Brice, Pauline; Malphettes, Marion

doi:10.1016/j.jaip.2019.04.039

Cited by 6 publications

(5 citation statements)

References 8 publications

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“…Symptoms (pruriginous skin lesions, angioedema, asthma, fasciitis, tenosynovitis) persisted in the three other patients, and dermatitis even worsened in two, with biopsies showing mixed eosinophilic and lymphocytic infiltrates. In the same line, one group reported complete absence of clinical response to mepolizumab in a patient with CD3 − CD4 + HES despite normalization of blood eosinophilia (36), and investigators at the NIH reported that only 2 of the 6 L-HES patients receiving mepolizumab on a compassionate use basis presented a complete response allowing for a reduction in OCS dosing (37). Similarly, among 4 subjects with CD3 − CD4 + T cells receiving benralizumab, a monoclonal anti-IL-5 receptor antibody, in the setting of a single-center pilot study, 3 relapsed within months after treatment initiation (38).…”

Section: Interleukin-5-targeted Therapymentioning

confidence: 89%

“…A recent single case report has shown a response to mycophenolic acid in a patient who had not responded to mycophenolate mofetil or peg-IFN-α ( 33 ). Alemtuzumab, a monoclonal anti-CD52 antibody able to target eosinophils in addition to the majority of leukocytes including lymphocytes, has been used anecdotally to treat CD3 − CD4 + HES ( 21 , 36 ) with an observed decrease in the CD3 − CD4 + T cell count in one case. Besides its limited availability, use of alemtuzumab in this setting is not justified given significant treatment-related morbidity ( 41 ).…”

Section: Discussionmentioning

confidence: 99%

“…ASCT should be considered early to treat T cell lymphoma arising in this setting as conventional chemotherapy is not effective. eosinophils in addition to the majority of leukocytes including lymphocytes, has been used anecdotally to treat CD3 − CD4 + HES (21,36) with an observed decrease in the CD3 − CD4 + T cell count in one case. Besides its limited availability, use of alemtuzumab in this setting is not justified given significant treatment-related morbidity (41).…”

Section: Disease Coursementioning

confidence: 99%

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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: Interleukin-5-targeted Therapymentioning

confidence: 89%

Section: Discussionmentioning

confidence: 99%

Section: Disease Coursementioning

confidence: 99%

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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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“…Fludarabine, low‐dose cyclophosphamide, romidepsin and dexpramipexole have each been reported to be successful in small numbers of patients with L‐HES. Dosing or adjunctive therapy was not specified for these cases 23,34,37,38 …”

Section: Resultsmentioning

confidence: 99%

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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“…188 Hydroxyurea and imatinib are less likely to demonstrate efficacy in this lymphocyte-variant of HE, compared to myeloproliferative forms of the disease which can be very responsive to these drugs as discussed above. The histone deaceytlase inhibitor romidepsin has been successfully used in refractory cases 189,190 Successful use of the JAK inhibitors tofacitinib and ruxolitinib has been reported in L-HES 191 and is being studied in a prospective clinical trial (NCT03801434). measurements in individuals with pulmonary disease have been observed with anti-IL-5 antibody therapy.…”

Section: Treatment Of Lymphocyte-variant Hypereosinophiliamentioning

confidence: 99%

World Health Organization and International Consensus Classification of eosinophilic disorders: 2024 update on diagnosis, risk stratification, and management

Shomali,

Gotlib

2024

American J Hematol

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Disease OverviewThe eosinophilias encompass a broad range of non‐hematologic (secondary or reactive) and hematologic (primary or clonal) disorders with the potential for end‐organ damage.DiagnosisHypereosinophilia (HE) has generally been defined as a peripheral blood eosinophil count greater than 1.5 × 109/L, and may be associated with tissue damage. After the exclusion of secondary causes of eosinophilia, diagnostic evaluation of primary eosinophilias relies on a combination of various tests. They include morphologic review of the blood and marrow, standard cytogenetics, fluorescence in situ hybridization, molecular testing and flow immunophenotyping to detect histopathologic or clonal evidence for an acute or chronic hematolymphoid neoplasm.Risk StratificationDisease prognosis relies on identifying the subtype of eosinophilia. After evaluation of secondary causes of eosinophilia, the 2022 World Health Organization and International Consensus Classification endorse a semi‐molecular classification scheme of disease subtypes. This includes the major category “myeloid/lymphoid neoplasms with eosinophilia and tyrosine kinase gene fusions” (MLN‐eo‐TK), and the MPN subtype, “chronic eosinophilic leukemia” (CEL). Lymphocyte‐variant HE is an aberrant T‐cell clone‐driven reactive eosinophila, and idiopathic hypereosinophilic syndrome (HES) is a diagnosis of exclusion.Risk‐Adapted TherapyThe goal of therapy is to mitigate eosinophil‐mediated organ damage. For patients with milder forms of eosinophilia (e.g., <1.5 × 109/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. Pemigatinib was recently approved for patients with relapsed or refractory FGFR1‐rearranged neoplasms. 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, is approved by the U.S Food and Drug Administration for patients with idiopathic HES. Cytotoxic chemotherapy agents, and hematopoietic stem cell transplantation have been used for aggressive forms of HES and CEL, with outcomes reported for limited numbers of patients. Targeted therapies such as the IL‐5 receptor antibody benralizumab, IL‐5 monoclonal antibody depemokimab, and various tyrosine kinase inhibitors for MLN‐eo‐TK, are under active investigation.

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Romidepsin is an effective and well-tolerated therapy in CD3−CD4+ lymphocyte-variant hypereosinophilic syndrome: A case report

Cited by 6 publications

References 8 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 and International Consensus Classification of eosinophilic disorders: 2024 update on diagnosis, risk stratification, and management

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