Zhao Yan, Cheng Ji
Cardiovascular Center, Tianjin Chlidren's Hospital, Tianjin, China.
Tianjin Chlidren's Hospital, 225 Machang Road, Tianjin, China.
J Interv Med. 2023 Jul 31;6(3):130-133. doi: 10.1016/j.jimed.2023.03.004. eCollection 2023 Aug.
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.
一名患有卡萨巴赫-梅里特现象(KMP)的婴儿,右下肢出现巨大皮下肿块,伴有严重低纤维蛋白原血症和血小板减少症。给予糖皮质激素,并辅以包括输血制品和凝血因子在内的支持治疗,以逆转致命的弥散性血管内凝血和急性溶血。糖皮质激素剂量缓慢递减,并加用西罗莫司治疗血管瘤。患者随后接受了介入治疗。经过6个月的药物和介入治疗,患者情况良好,血小板计数正常,肿瘤体积明显缩小,原发性皮肤病变变为浅粉红色。目前,患者仍在服用西罗莫司,随访6个月后未观察到血小板减少症复发或肿块进一步生长。