From hypereosinophilia to hypereosinophilic syndrome (HES): real-world
efficacy of the two-tails approach in improving HES diagnosis and
reducing diagnostic delay.
Abstract
Background. Hypereosinophilic Syndrome (HES) is a rare disorder with an
extremely heterogeneous clinical presentation, leading to diagnostic
delay, and a very bad prognosis if left untreated. Our study aimed at
highlighting the effectiveness and feasibility of a two-tails approach
in improving the HES diagnosis in an Italian Immunology excellence
university centre. Methods. A retrospective observational single-centre
study was conducted. All the patients underwent blood and instrumental
tests to highlight HES aetiology and early detect any organ damage.
Results: 79 patients (31 females, 39.2%), with a mean age of 54.9
years, were included in the study. 19 (24.1%) patients were diagnosed
with reactive HES, 15 (19.0%) with Overlap HES (EGPA), 1 (1.3%) with
myeloid-HES, 10 (12.7%) with lymphocytic HES and 8 (10.1%) with
idiopathic HES. 63 patients showed involvement of at least two organs:
the lung (32/63, 50.7%), the skin (24/63, 38.1%), the bowel (23/63,
36.5%), and the peripheral nervous system (25.4%). Eight patients
(8/63, 12.7%) showed a heart involvement. The diagnosis was achieved in
4±1.8 months and no deaths were observed. Conclusion. HE is a common
reason for allergists’ and clinical immunologists’ consultations, and
the two-tails approach should be implemented from the first evaluation
of patients with HE. This will aim at detecting early organ damage and
reducing both diagnostic delay and the risk for mistakes in the
classification of HES subtypes, thus allowing a prompt and tailored
treatment and better outcome.