Wang Zuopeng, Li Kai, Yao Wei, Dong Kuiran, Xiao Xianmin, Zheng Shan
Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, People's Republic of China.
Pediatr Blood Cancer. 2015 Apr;62(4):577-80. doi: 10.1002/pbc.25296. Epub 2014 Oct 24.
Kaposiform hemangioendothelioma (KHE) with Kasabach-Merritt phenomenon (KMP) still remains a particular and life-threatening disease. The purpose of this study was to evaluate the efficacy of vincristine (VCR) and the possibility of replacement with steroids in the treatment of steroid-resistant KHE with KMP.
We retrospectively reviewed the medical records of 37 patients with steroid-resistant KHE who were treated at the Children's Hospital of Fudan University between March 2003 and March 2013.
The age of initial diagnosis with KHE was between 1 day and 10 months. Eight and 29 cases were located in the superficial and deep soft tissues, respectively. Thirty-seven KHE lesions did not respond well to steroids before starting VCR treatment. Twenty-six KHE lesions achieved complete remission, with platelet counts reaching normal levels within7.6 ± 5.2 weeks after VCR treatment. The vascular tumor began to decrease in size or soften at an average of 4.9 ± 2.7 weeks. Two KHE lesions had partial responses and one remains in treatment. Eight KHE lesions had no apparent response to VCR and thus received other therapies. Twenty-eight patients have ended treatment with VCR; the average length of treatment was 31.2 ± 5.9 weeks. Side effects occurred in 48.6% of patients who received steroids, and in 11.4% of patients who received VCR treatment. The mean follow-up time was 3.5 years. No recurrences have been reported.
VCR appears to be a safe and effective treatment option in the management of steroid-resistant KHE with KMP, and recommended as first-choice treatment.
伴有卡萨巴赫-梅里特现象（KMP）的卡波西样血管内皮瘤（KHE）仍然是一种特殊且危及生命的疾病。本研究的目的是评估长春新碱（VCR）的疗效以及在治疗对类固醇耐药的伴有KMP的KHE中用类固醇替代的可能性。
我们回顾性分析了2003年3月至2013年3月在复旦大学附属儿科医院接受治疗的37例对类固醇耐药的KHE患者的病历。
KHE的初始诊断年龄在1天至10个月之间。8例和29例分别位于浅表和深部软组织。37个KHE病灶在开始VCR治疗前对类固醇反应不佳。26个KHE病灶实现完全缓解，VCR治疗后7.6±5.2周内血小板计数达到正常水平。血管肿瘤平均在4.9±2.7周开始缩小或变软。2个KHE病灶有部分反应，1个仍在治疗中。8个KHE病灶对VCR无明显反应，因此接受了其他治疗。28例患者已结束VCR治疗；平均治疗时长为31.2±5.9周。接受类固醇治疗的患者中有48.6%出现副作用，接受VCR治疗的患者中有11.4%出现副作用。平均随访时间为3.5年。未报告复发情况。
VCR似乎是治疗对类固醇耐药的伴有KMP的KHE的一种安全有效的选择，推荐作为首选治疗方法。
