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Article Dans Une Revue (Article De Synthèse) Clinical Immunology Année : 2023

JAK inhibition for CD3− CD4+ lymphocytic-variant hypereosinophilic syndrome

Résumé

Alternatives are urgently needed in patients with CD3- CD4+ lymphocytic-variant hypereosinophilic syndrome (L-HES) requiring high-level steroids or who are unresponsive and/or intolerant to conventional alternative therapies. We report five L-HES patients (44-66 years) with cutaneous involvement (n = 5) and persistent eosinophilia (n = 3) despite conventional therapies, who successfully received JAK inhibitors (tofacitinib n = 1, ruxolitinib n = 4). JAKi led to complete clinical remission in the first 3 months in all (with prednisone withdrawal in four). Absolute eosinophil counts normalized in cases receiving ruxolitinib, while reduction was partial under tofacitinib. After switch from tofacitinib to ruxolitinib, complete clinical response persisted despite prednisone withdrawal. The clone size remained stable in all patients. After 3-13 months of follow-up, no adverse event was reported. Randomized controlled trials are now mandatory to optimize the use of JAKi in L-HES.
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Dates et versions

hal-04019811 , version 1 (08-03-2023)

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Stanislas Faguer, Matthieu Groh, François Vergez, Mathilde Hunault-Berger, Nicolas Duployez, et al.. JAK inhibition for CD3− CD4+ lymphocytic-variant hypereosinophilic syndrome. Clinical Immunology, 2023, pp.109275. ⟨10.1016/j.clim.2023.109275⟩. ⟨hal-04019811⟩
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