Duan Lucy, Renzi Samuele, Weidman Danielle, Waespe Nicolas, Chami Rose, Manson David, Cada Michaela, Carcao Manuel
Departments of Paediatrics.
Paediatrics, Division of Haematology/Oncology.
J Pediatr Hematol Oncol. 2020 Jan;42(1):74-78. doi: 10.1097/MPH.0000000000001268.
Kaposiform hemangioendothelioma (KHE) is a rare infiltrative vascular tumor that may be associated with Kasabach-Merritt Phenomenon (KMP), which is a consumptive coagulopathy with potentially life-threatening thrombocytopenia. Management of KHE and KMP is challenging, and currently, there are no standardized validated treatment protocols. Mammalian target of rapamycin inhibitors have been shown to be effective in the treatment of KHE. We describe a term male who presented as a diagnostic dilemma with life-threatening pleural and pericardial effusions and severe thrombocytopenia. After extensive work-up the etiology for his condition was determined to be KHE with KMP. The patient was commenced on sirolimus and responded well to therapy with resolution of KMP.
卡波西样血管内皮瘤（KHE）是一种罕见的浸润性血管肿瘤，可能与卡萨巴赫-梅里特现象（KMP）相关，KMP是一种消耗性凝血病，可导致有潜在生命危险的血小板减少。KHE和KMP的治疗具有挑战性，目前尚无标准化的有效治疗方案。雷帕霉素靶蛋白抑制剂已被证明对KHE治疗有效。我们描述了一名足月男婴，他因危及生命的胸腔和心包积液以及严重血小板减少而面临诊断难题。经过全面检查，确定其病因是伴有KMP的KHE。患者开始使用西罗莫司治疗，对治疗反应良好，KMP得到缓解。
