Causes of hypereosinophilia in 100 consecutive patients. (18th May 2020)

• Record Type: Journal Article
• Authors: Moller, Daniel; Tan, Julia; Gauiran, Deonne Thaddeus V.; Medvedev, Nadia; Hudoba, Monika; Carruthers, Mollie N.; Dehghan, Natasha; van den Berghe, Janette; Bruyère, Helene; Chen, Luke Y.C.
• Is Part Of: European journal of haematology. Volume 105:Number 3(2020)
• Page Start: 292
• Page End: 301
• Publication Date: 2020-05-18
• Subjects: eosinophil -- hypereosinophilic syndrome -- IgG4‐related disease -- lymphocyte‐variant hypereosinophilic syndrome; Hematology -- Periodicals; Blood -- Diseases -- Periodicals; Blood -- Periodicals; 616.15005
• DOI: 10.1111/ejh.13437
• Languages: English
• ISSNs: 0902-4441

Abstract

Abstract: Background: Hypereosinophilia (HE, persistent peripheral blood eosinophilia > 1.5 × 10 9 /L) and hypereosinophilic syndrome (HES, HE with end‐organ damage) are classified as primary (due to a myeloid clone), secondary (due to a wide variety of reactive causes), or idiopathic. Diagnostic evaluation of eosinophilia is challenging, in part because secondary causes of HE/HES such as lymphocyte‐variant HES (L‐HES) and vasculitis are difficult to diagnose, and emerging causes such as immunoglobulin G4‐related disease (IgG4‐RD) have rarely been examined. Objective and Methods: We reviewed 100 consecutive patients with HE/HES who underwent extensive evaluation for primary and secondary eosinophilia at a single tertiary care center to determine causes of HE/HES in a modern context. Results: Six patients had primary HE/HES, 80 had a discrete secondary cause identified, and 14 had idiopathic HE/HES. The most common causes of secondary eosinophilia were L‐HES/HES of unknown significance (L‐HESus) (20), IgG4‐RD (9), and eosinophilic granulomatosis with polyangiitis (EGPA) (8). Conclusions: In contrast to other large published series of HE/HES, most patients in this study were found to have a discrete secondary cause of eosinophilia and only 14 were deemed idiopathic. These findings highlight the importance of extensive evaluation for secondary causes of eosinophilia such as L‐HES, IgG4‐RD, and EGPA.