PDF(Pubmed)
Abstract：
BACKGROUND: Kaposiform hemangioendothelioma is an aggressive vascular tumor that is often associated with life-threatening coagulopathies and Kasabach-Merritt phenomenon. Pathologic biopsies can provide a good basis for diagnosis and treatment. Therapy with srolimus combined with glucocorticoids may offer patients a favorable prognosis.
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.
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.
摘要：
背景：Kaposiform血管内皮瘤是一种侵袭性血管肿瘤，常伴有危及生命的凝血病和Kasabach-Merritt现象。病理活检可为诊断和治疗提供良好的依据。斯罗莫司联合糖皮质激素治疗可能为患者提供良好的预后。
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。
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。
