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An Unusual Hypereosinophilc Syndrome
Sunday, March 6, 2016
South Exhibit Hall H (Convention Center)
Massimo Arquati, Maddalena A. Wu, Roberto Castelli, Marco Cicardi
Rationale: Hypereosinophilic syndromes (HES) constitute a rare and heterogeneous group of disorders, defined as persistent and marked blood eosinophilia (> 1.5 × 109/L for more than six consecutive months) associated with evidence of eosinophil-induced organ damage, when other causes have been excluded

Methods: We describe the case of a young patient affected by an uncommon HES

Results: A 34-year-old Caucasian  man referred to our Hospital because of recurrent edema (periodic angioedema?) of the extremities associated with significant weight gain and pruritus leading to chronic administration of steroids with partial relief of symptoms. No effects were observed on eosinophil levels which otherwise presented great variations depending on time and temperature of collecting samples. Laboratory tests confirmed persistent hypereosinophilia associated with elevated IgM, polyclonal IgM-cryoglobulinemia and increased levels of IL-5 (not related with eosinophils count), ECP and tryptase. Bone marrow aspiration and biopsy with cytogenetic (FISH) analyses were negative for chromosomal rearrangements. There was no evidence of clonally expanded T cell population. Ultrastructural analysis of eosinophils on blood sample and skin biopsy demonstrated aspecific abnormalities of eosinophil granules. Extensive investigations failed to reveal an underlying disease known to be associated with increased eosinophil count. Moreover, organ damage and/or dysfunction related to eosinophilic infiltration were excluded.

Conclusions: Although the diagnosis of Gleich syndrome is possible, this appears to be a complex clinical case deserving further investigation. We speculate that the fluctuations of eosinophil levels detected by automated counters and their dependence on time and temperature might suggest a possible role for cryoglobulins, inducing eosinophil morphologic alteration or aggregation.