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机构地区:[1]Cardiovascular Center,Tianjin Chlidren's Hospital,Tianjin,China [2]Tianjin Chlidren's Hospital,225 Machang Road,Tianjin,China
出 处:《Journal of Interventional Medicine》2023年第3期130-133,共4页介入医学杂志(英文)
摘 要:An infant with Kasabach-Merritt Phenomenon(KMP)presented with a giant subcutaneous mass in the right lower limb,severe hypofibrinogenemia,and thrombocytopenia.Glucocorticoids,along with supportive treatments including transfusion of blood products and clotting factors,were administered to reverse fatal disseminated intravascular coagulation and acute hemolysis.The glucocorticoid dose was tapered slowly,and sirolimus was added to treat the hemangiomas.The patient subsequently underwent interventional therapy.After 6 months of medical and interventional therapy,the patient was doing well with a normal platelet count,the tumor volume was markedly reduced,and the primary cutaneous lesion became pale pink.Currently,the patient remains on sirolimus,and no recurrence of thrombocytopenia or further growth of the mass was observed after six months of follow-up.
关 键 词:Kasabach-Merritt phenomenon(KMP) Kaposiform hemangioendothelioma(KHE) SIROLIMUS Interventional therapy
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