激素联合长春新碱和栓塞硬化剂治疗婴幼儿脉管肿瘤伴Kasabach-Merritt现象

摘要

目的 总结和探讨婴幼儿脉管肿瘤伴Kasabach-Merritt现象的治疗方法,并评价药物联合栓塞硬化疗法的效果.方法 回顾性分析2007年2月至2014年11月接受药物联合栓塞硬化治疗的25例婴幼儿脉管肿瘤伴Kasabach-Merritt现象的临床资料,所有患儿均接受甲泼尼龙口服,每天2 mg/kg,2～3周后血小板计数无明显回升者联合长春新碱静脉用药,每周1.5 mg/m2;患儿血小板计数接近正常后,以无水乙醇(1 ～3 ml/次)、1％聚桂醇(1.25 ～5 ml/次)及0.7％复方倍他米松(0.25 ～1 ml/次)行栓塞硬化治疗,平均4.56次/例.随访9个月至6.5年,平均42个月,统计分析凝血指标变化和瘤体消退情况,随访中观察瘤体有无复发,患儿生长发育及肢体功能等.结果 25例患儿中16例单独应用激素4～7周,9例应用激素无效后联合应用长春新碱2～5周,血小板计数均得以明显回升,平均(94.3±18.5)×109/L.期间11例患儿接受血小板输注,其中3例辅以丙种球蛋白输注.患儿首次入院接受1～4次栓塞硬化治疗,每例平均2.6次,治疗结束后1周血小板计数回升至(167 ～312)×109/L,平均(258.5±34.4)×109/L.血红蛋白及凝血功能1～5周逐渐升至正常,患儿精神状态改善,食欲增强,体重增加,哭闹不安情绪缓解,出血现象消退.13例瘤体在3～12个月内消失,12例在13 ～ 36个月内消失.长期随访中仅有1例血小板再次降低,栓塞硬化治疗后恢复,所有患儿体重、身高、免疫力等同正常儿童发育,运动功能良好.结论 糖皮质激素、长春新碱和栓塞硬化的联合应用能够有效地减少瘤体对血小板的捕获,控制病情进展,并降低单独用药的不良反应.%Objective To summarize the management of infant vascular tumors with KasabachMerritt phenomenon (KMP) and to evaluate the effect of drug combined with sclerotherapy.Methods From Feb.2007 to Nov.2014,25 cases with KMP,who underwent drug therapy combined with sclerotherapy,were retrospectively studied.Oral corticosteroids (2 mg/kg per day) was used as the firstline therapy on all of the patients and intravenous vincristine (1.5 mg/m2 every week) was added when the platelet counts didn't recover obviously after 2-3 weeks.After the recovery of the platelet counts,the patients were admitted for sclerotherapy (average,4.56 sessions per case)with 100％ alcohol (1-3 ml per session),Lauromacrogol (1.25-5 ml per session) and betamethasone (0.25-1 ml per session).All the patients were followed up for 42 months (range,9 months to 6.5 years).Therapeutic outcomes were assessed by evaluating platelet counts,size of lesion,function of trunk and limb.Results All the 25 cases got obvious recovery in the platelet counts [average,(94.3 ± 18.5) × 109/L] after drug therapy,of which 16 were treated by single oral corticosteroids for 4-7 weeks and 9 were treated by corticosteroids plus intravenous vincristine for 2-5 weeks.Meantime,11 cases received platelet transfusions,of which 3 were coupled with gamma globulin intramuscularly.During the first admission,each of the 25 cases received 1-4 sessions of sclerotherapy (average,2.6 sessions each case).One week after the sclerotherapy,the platelet counts returned to (167-312) × 10 9/L(average,(258.5 ± 34.4) × 109/L).The hemoglobin and blood coagulation function returned to normal within 1-5 weeks.Meanwhile the mental condition,appetite,body weight,sleeping were greatly improved.The size of the lesions decreased gradually after the combined therapy including 13 cases within 3-12 months and 13 cases within 13-36 months.Long term follow-up indicated that only 1 case need treatment for recurrent decrease of platelet counts,and all of the 25 cases kept the normal weight,height,immunity as well as the growing development.Conclusions Oral corticosteroids plus intravenous vincristine combined with sclerotherapy is a reliable management with high cure rate,short course and minor side-effect.