Winning Abstracts from the 2011 Medical Student Abstract Competition: Idiopathic Hypereosinophilic Syndrome Masquerading as False Positive Strongyloides Stecoralis
Author: Laura E Divoky, MD, Northeast Ohio Medical University, Class of 2011, Summa Health System, PGY-1
Introduction: Hypereosinophilic syndrome (HES) is a heterogeneous group of conditions characterized by blood eosinophilia and end-organ dysfunction from eosinophilic infiltration and activation, thrombotic events and toxic mediator release.1-3 End-organ damage commonly affects the skin, heart, lungs, gastrointestinal tract, joints, and nervous system. Idiopathic HES is defined by persistent elevation of blood eosinophil count greater than 1500/碌L for six consecutive months, of an unknown cause, and evidence of underlying end-organ damage.1-3
Case Report: A 50-year-old Caucasian female with multiple food sensitivities and atopy presents with chronic dysphagia, abdominal cramping and watery diarrhea. Her medication and travel history were unremarkable. Lab values were notable for persistently elevated eosinophil count of 1,909 cells/碌L, elevated IgE levels of 479 IU/mL (normal 0.0-100.0) and elevated Strongyloides stecoralis IgG titers of 7.65 IV (normal < 2.11). Upper endoscopy with biopsies was performed and histopathologic exam revealed florid eosinophilic inflammation with eosinophilic cryptitis pervading the esophagus and stomach. Duodenal biopsies were negative for ova and parasitic infiltration. Despite Ivermectin therapy, the Strongyloides titers remained elevated, with eosinophil count increased to 2,503 cells/碌L. It was concluded that the elevated titers represented a false positive and parasitic infection was excluded as a source for her eosinophilia. Bone marrow biopsy revealed no evidence of a clonal T- or B-cell population on immunophenotyping, and FIP1L1-PDGFRA gene fusion was not detected.
Discussion: This case represents a unique diagnostic challenge because no apparent cause for the eosinophilia with gastrointestinal involvement could be identified, despite an extensive workup that ruled out clonal and reactive eosinophilia (i.e. infections, allergies, respiratory diseases, vasculitides, mixed connective tissue disease, medications, and non-hematological malignancies). Despite strict allergen avoidance, eosinophilia did not resolve. Despite the unique presentation, we surmise that this patient meets the criteria for IHES and warrants close monitoring with prompt treatment with prednisone or interferon-alfa to minimize end-organ damage from eosinophilia.
References:
- Chusid MJ, Dale DC, West BC, Wolff SM. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore). 1975;54(1):1-27.
- Brito-Babapulle F. The eosinophilias, including the idiopathic hypereosinophilic syndrome. Br J Haematol. 2003;121(2):203-223.
- Tefferi A. Blood eosinophilias: a new paradigm in disease classification, diagnosis, and treatment. Mayo Clin Proc. 2005;80(1):75-83.