Steroid-unresponsive hypereosinophilic syndrome

Abstract

Hypereosinophilic syndrome in children is rare and is characterised by sustained over-production of eosinophils. An 11-year-old boy was diagnosed with hypereosinophilic syndrome as per standard criteria. He was treated initially with intravenous methylprednisolone, followed by oral prednisolone, to which he showed a limited response. The highest absolute eosinophil count was 27.0×10(9)/L. He was commenced on imatinib mesylate but, because of no response, he was switched to hydroxyurea combined with low-dose prednisolone to which he showed improvement with decreasing levels of eosinophils.

A Case Report of Eosinophilic Esophagitis Accompanying Hypereosinophilic Syndrome

“…Our patient had all three of the diagnostic characteristics with involvement of the esophagus, which is a rare finding. The pharmacologic options for management of HES include tyrosine kinase inhibitors in those with 4q12 deletion and other drugs like glucocorticoids [7], interferon alpha [8], and chemotherapeutic agents, such as hydroxyurea [9]. Our patient responded very well to corticosteroid therapy and showed marked improvement in her symptoms.…”

Rapidly evolving asymptomatic eosinophilia in a patient with lung adenocarcinoma causes cognitive disturbance and respiratory insufficiency: Case report

“…However, a number of patients are non-responsive to corticosteroids, but respond well to hydroxyurea (16). Hydroxyurea is also reported to be an effective first-line agent in hypereosinophilic syndrome (15).…”

Síndrome hipereosinofílico y síndrome de Churg-Strauss ¿Espectro de una misma enfermedad? Nuevos conceptos

“…Se considera que la tasa de supervivencia es de más de 80% para los pacientes que responden a los corticoste- roides (CCS); sin embargo, las recaídas son comunes y a veces es necesario administrarlos por tiempos prolongados o cambiar a otro tratamiento (96). Si se requieren dosis de prednisona mayores a 10 mg/d de mantenimiento o las recaídas son muy constantes, hidroxiurea o interferón alfa son opciones que se puede adicionar (97) y para los pacientes que no responden a CSS o deben ser suspendidos por sus efectos adversos ciertas moléculas, como ciclosporina A, mepolizumab, alemtuzumab, reslizumab y recientemente el anticuerpo contra la cadena alfa del receptor de la IL-5 (MEDI-563), han demostrado ser beneficiosas (50,(98)(99)(100)(101)(102)(103)(104).…”