야간뇨와 호흡곤란으로 발현된 신장을 침범한 특발성 호산구 증가 증후군 1예
A Case of Idiopathic Hypereosinophilic Syndrome Presenting with Nocturia and Dyspnea
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Idiopathic hypereosinophilic syndrome (HES) is characterized by marked (＞1,500/μL) and persistent (＞6months) 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-α(FIP1L1-PDGFRα) gene rearrangement on BM aspiration. He was treated with oral glucocorticoid and tyrosine kinase inhibitor (imatinib mesylate). His symptoms were improved and proteinuria disappeared.
Abstract
Idiopathic hypereosinophilic syndrome (HES) is characterized by marked (＞1,500/μL) and persistent (＞6months) 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-α(FIP1L1-PDGFRα) gene rearrangement on BM aspiration. He was treated with oral glucocorticoid and tyrosine kinase inhibitor (imatinib mesylate). His symptoms were improved and proteinuria disappeared.
참고문헌 (15)
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