- 期刊
Kaposiform Hemangioendothelioma Presenting with Kasabach-Merritt Syndrome
併發Kasabach-Merritt症候群之類卡波西氏血管內皮細胞瘤
摘要
Kasabach-Merritt症候群自1940年首例報告之後，累計至今的病例報告數不超過250例。臨床上診斷症候群的標準為：血管瘤合併血小板減少，貧血及瀰漫性血管內凝血異常。併發症候群的血管瘤種類在過去未有人深究過，直到1993年Zukerburg等人才發現一般血管瘤並不會引起Kasabach - Merritt症候群；會併發此症候群的只有類卡波西氏血管內皮細胞瘤(Kaposiform hemangioendothelioma)及毛叢狀血管瘤(Tufted angioma)。此二種腫瘤在臨床及病理學上皆與一般的血管瘤大不相同而擁有獨樹一幟的特色。臨床上正確的診斷對於治療方針擬定有決定性的影響。
關鍵字
無資料並列摘要
The double eponym Kasabach-Meritt has been used since the case report in 1940 for a clinical presentation of giant hemangioma with thrombocytopenia, petechia, and bleeding. However, recently two retrospective studies revealed that KMS is not associated with hemangiomas but rather with KHE or tufted angioma (TA). Kaposiform hemangioendothelioma (KHE) is a pathologically benign and clinically aggressive vascular neoplasm in children which was first described by Zukerburg et al. in 1993. We present a case of KHE manifested with KMS and the pathological picture of KHE.
延伸閱讀
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