난치성 편측 흉수와 불응성 혈소판 감소증으로 나타난 흉강 내 혈관내피종 1예

등록일 2017.01.06 파일확장자어도비 PDF (pdf) | 5페이지 | 가격 4,000원
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발행기관 : 대한천식알레르기학회(구 대한알레르기학회) 수록지정보 : Allergy asthma & respiratory disease / 4권 / 6호
저자명 : 김효원 ( Hyo Won Kim ) , 최윤정 ( Yun Jung Choi ) , 홍경택 ( Kyung Taek Hong ) , 강형진 ( Hyoung Jin Kang ) , 박경덕 ( Kyung Duk Park ) , 김세희 ( Sehui Kim ) , 최영훈 ( Young Hoon Choi ) , 김우선 ( Woo-sun Kim ) , 서동인 ( Dong In Suh )

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A variety of diseases are associated with the development of unilateral pleural effusion. Although unilateral pleural effusion is common, refractory unilateral pleural effusion is rare. It is important to make an accurate diagnosis using proper diagnostic tools. Thrombocytopenia is one of the rare conditions occurring from various diseases such as severe infection or autoimmune diseases. It can be life-threatening if accurate diagnosis and treatment are delayed and be a clue to accurate diagnosis in differential diagnosis from refractory pleural effusion. Kasabach-Merrit syndrome (KMS) is often accompanied by extensive vascular tumors and characterized by consumptive coagulopathy with profound thrombocytopenia. It is also important to have a high index of suspicion for the diagnosis. We report a case of KMS in a 2-month-old female infant with a vascular tumor on her left intrathoracic cage, who had presented refractory unilateral pleural effusion and thrombocytopenia. Initially, the patient was diagnosed as having complications of severe infection, and a chest tube was inserted for aggressive treatment. However, her unilateral pleural effusion persisted, and thrombocytopenia and hypofibrinogenemia were refractory. Chest imaging revealed an infiltrating large vascular tumor involving the cardiac border, diaphragm, and chest wall. The patient`s unilateral pleural effusion was misidentified as an infectious condition at the initial stage. As a result of the ultrasonography-guided biopsy, it was revealed to be Kaposiform hemangioendothelioma. The patient was cured after treatment for KMS. (Allergy Asthma Respir Dis 2016:4:453-457)

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