Idiopathic hypereosinophilic syndrome: a rare but fatal condition presenting with common symptoms.
BACKGROUND
Idiopathic hypereosinophilic syndrome (IHES) is a leukoproliferative disorder characterized by cytokine-induced overproduction of eosinophils with resultant multiorgan infiltration and damage. The diagnostic criteria includes evidence of end organ damage, exclusion of all other causes of eosinophilia and sustained absolute eosinophil count (AEC) > 1,500 cells/mcl for at least 6 months.


METHODS
An 88-year-old Caucasian female presented with persistent severe chronic cough, weight loss and rhinorrhea unresponsive to various treatments. Her workup during admission revealed absolute eosinophil count of 17,447 and bone marrow biopsy showed eosinophilia with no lymphoproliferative process. After excluding all other causes of eosinophilia, a diagnosis of IHES was made and prednisone was started. Symptoms resolved and her absolute eosinophil count progressively decreased.


CONCLUSIONS
IHES mimics several other diseases. Mild eosinophilia should be worked up irrespective of age and sex. A high index of suspicion is required for earlier diagnosis and treatment, which could reduce morbidity and mortality.
Osowo
Ayodele
A
Department of Medicine, Unity Health System, Rochester, NY, USA. ayodeleo@hotmail.com
Fetten
James
J
Navaneethan
Sankar
S
D000284:Administration, Oral; D000369:Aged, 80 and over; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D003937:Diagnosis, Differential; D018450:Disease Progression; D004804:Eosinophils / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000175:diagnosis* / Q000188:drug therapy; D011241:Prednisone / Q000008:administration & dosage / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed
D005938:Glucocorticoids; D011241:Prednisone