Back to Search Start Over

Prognostic factors of idiopathic hypereosinophilic syndrome: A nationwide survey in Japan.

Authors :
Honda, Akira
Masuda, Yasutaka
Oyama, Yu
Matsuda, Kensuke
Mizuno, Hideaki
Saito, Akiko M.
Katayama, Yoshio
Komatsu, Norio
Toyama, Kazuhiro
Kurokawa, Mineo
Source :
British Journal of Haematology. May2024, p1. 11p. 3 Illustrations, 6 Charts.
Publication Year :
2024

Abstract

Summary Idiopathic hypereosinophilic syndrome (iHES) is a condition wherein persistent hypereosinophilia associated with end‐organ damage occurs without any known causes. Due to the rarity of the disease, insufficient knowledge has been accumulated. We therefore conducted a retrospective, multicentre, nationwide survey on iHES in Japan. A total of 57 patients were identified. For 43 patients who received any treatment, all cases were first treated with corticosteroids. An eosinophil percentage of less than 30% in the bone marrow and the absence of oedema were identified as factors associated with steroid dependency. The 5‐year overall survival was 88.2%, and five patients died during follow‐up; factors associated with worse overall survival were age >50, haemoglobin <12 g/dL, activated partial thromboplastin time >34 s, the presence of dyspnoea, the presence of thrombotic tendency and the presence of renal failure. Given the rarity of fatalities in our cohort, time‐to‐next‐treatment (TTNT) was further analysed; the presence of renal failure, splenomegaly and lung abnormalities were associated with worse TTNT. Our nationwide study not only demonstrated clinical characteristics and the outcome of patients with iHES but also for the first time revealed clinical factors associated with steroid dependency and duration of first‐line corticosteroid efficacy. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
00071048
Database :
Academic Search Index
Journal :
British Journal of Haematology
Publication Type :
Academic Journal
Accession number :
177418648
Full Text :
https://doi.org/10.1111/bjh.19527