Korean J Asthma Allergy Clin Immunol.  2010 Sep;30(3):241-245.

A Case of Idiopathic Hypereosinophilic Syndrome Presenting with Nocturia and Dyspnea

Abstract

Idiopathic hypereosinophilic syndrome (HES) is characterized by marked (>1,500/microgliter) and persistent (>6 months) eosinophilia without an identifiable underlying cause. This condition is associated with multiorgan involvements. Renal involvement is rarely reported and little is known about renal morphologic changes in this disease. We report a case of HES with renal involvement presenting with nocturia and dyspnea. A 30-year-old man visited to our institution due to nocturia and dyspnea. Peripheral blood tests showed an increased eosinophil count (14,868/mm3). Chest CT showed extensive areas of ground-glass opacities in both lungs. Renal biopsy showed thrombotic microangiopathic nephropathy. Based upon his medical records, physical examination findings and laboratory data, he was diagnosed as having idiopatic HES with renal involvement. We found FIP1-like-1-platelet-derived growth factor receptor-alpha (FIP1L1-PDGFR alpha) gene rearrangement on BM aspiration. He was treated with oral glucocorticoid and tyrosine kinase inhibitor (imatinib mesylate). His symptoms were improved and proteinuria disappeared.

MeSH Terms

Adult
Biopsy
Dyspnea
Eosinophilia
Eosinophils
Gene Rearrangement
Hematologic Tests
Humans
Hypereosinophilic Syndrome
Lung
Medical Records
Nocturia
Physical Examination
Protein-Tyrosine Kinases
Proteinuria
Thorax
Protein-Tyrosine Kinases