16例婴幼儿卡梅现象临床疗效分析

Clinical Analysis of 16 Infants with Kasabach-Merritt Phenomenon

目的:总结16例卡梅现象患儿的临床治疗过程,为该病提供诊疗方案。方法:收集我科在2016年5月至2020年1月期间收治并确诊为卡梅现象的16例患儿(男13例、女3例)的临床资料并进行回顾性分析。结果:患儿在住院和门诊期间接受药物(糖皮质激素、长春新碱、西罗莫司)、介入栓塞、微波消融术和瘤内硬化剂注射等治疗。5例(31.2%)患儿治疗显效,随访2-3个月。其余患者随访6个月~36个月,5例(31.2%)治愈,3例(18.8%)好转,3例(18.8%)治疗有效尚在继续口服西罗莫司治疗中。结论:对于卡梅现象应在早期实施个体化治疗,介入栓塞术对供血动脉的肿物有显著疗效,糖皮质激素和长春新碱有助于早期控制病情进展,西罗莫司单独或联合治疗均获得显著疗效,微波消融术疗效好且微创,瘤内硬化剂注射治疗具有一定疗效,均可用于辅助治疗。

Objective: To summarize the clinical data of 16 infants with Kasabach-Merritt phenomenon, and provide plans for diagnosis and treatment. Methods: From May 2016 to January 2020, 16 children diagnosed as Kasabach-Merritt Phenomenon were admitted to our unit, including 13 boys and 3 girls. All clinical data were collected for retrospective analysis. Results: The patients received drug therapy(glucocorticoid, vincristine, sirolimus), interventional embolization, microwave ablation and intratumor sclerosant injection during inpatient and outpatient care. 5(31.2%) patients were treated effectively and followed up for 2 months to 3 months. The rest of the patients were followed up for 6 months to 36 months, five cases(31.2%) were cured, three cases(18.8%) were improved, and three cases(18.8%) were effective and still continued oral sirolimus. Conclusions: Early individual and comprehensive therapy should be adopted for Kasabach-Merritt phenomenon. Interventional embolization has a significant effect on tumors with clear blood supply arteries. Glucocorticoid and vincristine are helpful to control the progression of the disease. Sirolimus alone or in combination therapy provides significant effect. Minimally invasive microwave ablation and intratumor sclerosant injection have good effects and can be used as an adjuvant therapy.