반복적인 부비동염으로 내원한 Good 증후군 1예
A Case of Good’s Syndrome
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초록
Good 증후군은 흉선종과 면역결핍이 동반되는 질환으로, B세포의 감소로 인한 범저감마글로불린혈증과 CD4＋ T세포의 감소로 인한 세포성 면역저하가 동시에 나타나는 것을 특징으로 한다. 본 증례는 18개월 전부터 발생한 반복적인 부비동염을 주소로 내원한 43세 남자 환자로, 내원 1년 전 흉선종절제수술을 시행받았으며, 면역학적 검사상 범저감마글로불린혈증과 T세포의 감소 등의 이상 소견이 발견되었다. 정주 면역글로불린과 경험적 항생제(amoxicillin/clavulanic acid) 사용 후 부비동염이 호전되었으며, 예방접종과 정기적인 면역글로불린 투여 후 특이 합병증 없이 경과 관찰 중이다.
Abstract
Good's syndrome is the association of thymoma with immunodeficiency, characterized by hypogammaglobulinemia, B-cell lymphopenia and variably defects in cellular immunity with CD4＋ T-cell lymphopenia and an inverted CD4＋:CD8＋ T-cell ratio. We report a 43-year-old male patient who presented with a 18-month history of productive cough and postnasal drip. One year ago, he underwent the operation for resection of a thymoma. Despite of appropriate management, sinusitis relapsed multiple times. He was found to have hypogammaglobulinemia with nearly absent B cells(4/μL). The CD4＋ T-cell count was 554/μL with an inverted CD4＋:CD8＋ T-cell ratio of 0.6. His symptoms and signs improved with antibiotic treatment and monthly administration of intravenous immunoglobulin (IVIG, 400 mg/kg).
참고문헌 (14)
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