JAK inhibition for CD3- CD4+ lymphocytic-variant hypereosinophilic syndrome.
Clin Immunol
; 251: 109275, 2023 06.
Article
em En
| MEDLINE
| ID: mdl-36870379
ABSTRACT
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 (44-66â¯years) with cutaneous involvement (nâ¯=â¯5) and persistent eosinophilia (nâ¯=â¯3) despite conventional therapies, who successfully received JAK inhibitors (tofacitinib nâ¯=â¯1, ruxolitinib nâ¯=â¯4). JAKi led to complete clinical remission in the first 3â¯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 3-13â¯months of follow-up, no adverse event was reported. Prospective clinical trials are warranted to examine the use of JAKi in L-HES.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Síndrome Hipereosinofílica
Tipo de estudo:
Clinical_trials
/
Observational_studies
Limite:
Humans
Idioma:
En
Revista:
Clin Immunol
Assunto da revista:
ALERGIA E IMUNOLOGIA
Ano de publicação:
2023
Tipo de documento:
Article