Shen Weiming, Cui Jie, Chen Jianbin, Zou Jijun, Ji Yi, Chen Haini
Department of Plastic Surgery, Nanjing Children's Hospital, Nanjing Medical University, China.
Ann Plast Surg. 2010 Sep;65(3):361-3. doi: 10.1097/SAP.0b013e3181bfba1e.
Kasabach-Merritt syndrome is a life-threatening and localized consumption coagulopathy, characterized with profound thrombocytopenia and microangiopathic anemia. The huge tumor is the major cause of rapid platelet destruction, so we supposed the reduction of tumor size could reduce the platelet destruction and improve the clinical condition. In our cases, the vascular tumor occupied one of the extremities, or the head or face. However, removal of the whole tumor would have resulted in the amputation of this extremity or the destruction of the face, and partial tumor removal was suitable. The wound could be repaired with skin graft. Vincristine chemotherapy after the operation was necessary to prevent the enlargement of the remaining tumor. Two cases which were ineffectively treated by other means were treated in this way.
卡萨巴彻-梅里特综合征是一种危及生命的局限性消耗性凝血病,其特征为严重血小板减少和微血管病性贫血。巨大肿瘤是血小板迅速破坏的主要原因,因此我们推测肿瘤体积缩小可减少血小板破坏并改善临床状况。在我们的病例中,血管性肿瘤占据四肢之一、头部或面部。然而,切除整个肿瘤会导致该肢体截肢或面部毁损,因此适合进行部分肿瘤切除。伤口可用皮肤移植修复。术后进行长春新碱化疗对于防止残留肿瘤增大是必要的。有两例经其他方法治疗无效的病例就是采用这种方法治疗的。