Department of Aesthetic, Plastic, and Burn Surgery, Shandong Provincial Hospital, Shandong University, 324, Jingwu Road, Jinan, Shandong 250021, China; Department of Hemangioma and Vascular Malformation Surgery, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, 7 Weiwu Road, Zhengzhou 450003, China.
Department of Hemangioma and Vascular Malformation Surgery, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, 7 Weiwu Road, Zhengzhou 450003, China.
Clin Chim Acta. 2018 Nov;486:199-204. doi: 10.1016/j.cca.2018.07.044. Epub 2018 Aug 7.
To analyze the clinical characteristics and treatment of pediatric tufted angiomas(TA)complicated by Kasabach-Merritt Phenomenon (KMP).
A retrospective analysis was conducted on the clinical data and follow-up data of 13 patients diagnosed with TA complicated by KMP. Five male and 8 female patients with an average age of 5.7 months (range, 29 days to 1 year) were treated with surgery between January 2009 and June 2012. According to the size and location of lesions and the degree of thrombocytopenia, complete or subtotal resection was performed. The median follow-up period was 3.4 years (range, 1.7 years to 5.2 years). Therapeutic outcomes were evaluated by platelet count and lesion size.
Curative treatment of KMP is defined as restoration of normal hemostasis and elimination of tumor cells. Twelve patients achieved curative treatment and one died of multiple organ failure after operation. Ten patients received complete resection and three patients received incomplete resection. Thrombocyte count, hemoglobin and blood coagulation were respectively restored to normal levels within 1-3 days and 1-2 weeks post complete resection operation. One of the three patients who received subtotal resection operation died. In the other two patients, the platelet count fluctuated over time but remained above 60 × 10 /L, a significantly higher level than the preoperational level. Residual lesions slowly disappeared after continuous medication 3-6 months post operation.
Early surgical treatment of patients with TA complicated with KMP resulted in significantly higher curative rate and reduced side-effects of drugs.
分析儿童丛状血管瘤（TA）合并卡-梅现象（KMP）的临床特点及治疗方法。
回顾性分析 2009 年 1 月至 2012 年 6 月收治的 13 例 TA 合并 KMP 患儿的临床资料及随访资料。男 5 例，女 8 例；年龄 5.7 个月（29 天至 1 岁）。根据病变大小、部位及血小板减少程度，采用完全或大部分切除术。中位随访时间 3.4 年（1.7~5.2 年）。根据血小板计数及瘤体大小评估治疗效果。
KMP 的治愈标准为血小板恢复正常、瘤体消失。12 例患儿达到治愈标准，1 例患儿术后因多器官功能衰竭死亡。10 例患儿行完全切除术，3 例患儿行大部分切除术。完全切除术后 13 d，血小板计数、血红蛋白及凝血功能恢复正常，12 周内均恢复正常。3 例行大部分切除术的患儿中，1 例死亡，另 2 例血小板计数随时间波动，但均高于术前水平，且残留病变在术后 3~6 个月继续药物治疗过程中逐渐消退。
早期手术治疗 TA 合并 KMP 可显著提高治愈率，减少药物不良反应。
Zotero 插件