METHODS: A large purplish-red mass on the knee of a child with extremely progressive thrombocytopenia and refractory coagulation abnormalities. Conventional doses of glucocorticoids alone failed to improve coagulation abnormalities and the child developed large cutaneous petechiae and scalp hematomas.
METHODS: Kaposiform hemangioendothelioma combined with Kasabach-Merritt phenomenon.
METHODS: The patient received prednisolone 2.0 mg/kg*d for 4 days. Blood products were transfused to ensure vital signs and to complete the pathologic biopsy. Sirolimus combined with prednisolone was given after clarifying the diagnosis of Kaposiform hemangioendothelioma.
RESULTS: The tumor basically disappeared on examination and the ultrasound showed a subcutaneous hyperechoic mass with normal blood flow.
CONCLUSIONS: Sirolimus combined with glucocorticoids is effective in controlling Kasabach-Merritt phenomenon and pathologic biopsy is important for definitive diagnosis.
方法:1例儿童膝关节上有一个巨大的紫红色肿块,伴有极进行性血小板减少和难治性凝血异常。单独使用常规剂量的糖皮质激素无法改善凝血异常,并且孩子出现了大的皮肤瘀斑和头皮血肿。
方法:Kaposiform血管内皮瘤合并Kasabach-Merritt现象。
方法:患者接受泼尼松龙2.0mg/kg*d治疗4天。输血以确保生命体征并完成病理活检。明确Kaposiform血管内皮瘤的诊断后,给予西罗莫司联合泼尼松龙治疗。
结果:检查肿瘤基本消失,超声显示皮下高回声肿块,血流正常。
结论:西罗莫司联合糖皮质激素可有效控制Kasabach-Merritt现象,病理活检对明确诊断具有重要意义。