CAJ | 학술논문

目的评价长春新碱(vincristine,VCR)治疗卡波西样血管内皮瘤(Kaposiform hemangio endothelioma,KHE)伴K-M现象(Kasabach-Merritt phenomenon,KMP)的效用.方法回顾性分析我院2003年3月至2013年3月33例诊断为KHE伴KMP且激素治疗不敏感改用VCR治疗的患儿的临床资料并评估疗效、副作用及预后.33例患儿发病年龄1 d～10个月.男19例,女14例.浅部KHE 7例,深部KHE26例.33例均有血小板减少,25例同时伴有凝血功能异常.结果每例患儿前期均用激素治疗,但因病情反复或无效,改用VCR治疗.其中22例治愈,VCR治疗后血小板计数升至100×109/L以上平均时间(7.5±5.2)周,肿块开始软化或缩小时间为(4.9±2.6)周.2例显效,1例仍在治疗.8例无明显疗效或病情反复,改用其他治疗.24例VCR治疗患儿已停药,平均治疗周期为(31.1±6.0)周,平均随访时间3.7年,最长随访7.1年,无复发.结论激素治疗KHE伴KMP疗效有限,VCR治疗该病具有较好的安全性和有效性,对于激素无反应的患儿,建议改用VCR治疗.
목적 평개장춘신감(vincristine,VCR)치료잡파서양혈관내피류(Kaposiform hemangio endothelioma,KHE)반K-M현상(Kasabach-Merritt phenomenon,KMP)적효용.방법 회고성분석아원2003년3월지2013년3월33례진단위KHE반KMP차격소치료불민감개용VCR치료적환인적림상자료병평고료효、부작용급예후.33례환인발병년령1 d～10개월.남19례,녀14례.천부KHE 7례,심부KHE26례.33례균유혈소판감소,25례동시반유응혈공능이상.결과 매례환인전기균용격소치료,단인병정반복혹무효,개용VCR치료.기중22례치유,VCR치료후혈소판계수승지100×109/L이상평균시간(7.5±5.2)주,종괴개시연화혹축소시간위(4.9±2.6)주.2례현효,1례잉재치료.8례무명현료효혹병정반복,개용기타치료.24례VCR치료환인이정약,평균치료주기위(31.1±6.0)주,평균수방시간3.7년,최장수방7.1년,무복발.결론 격소치료KHE반KMP료효유한,VCR치료해병구유교호적안전성화유효성,대우격소무반응적환인,건의개용VCR치료.
Objective To evaluate the efficacy of vincristine (VCR) in the treatment of steroidresistant patients of kaposiform hemangioendothelioma (KHE) with Kasabach-Merritt phenomenon (KMP).Methods Retrospective analyses were conducted for the clinical data of 33 steroid-resistant cases of KHE with KMP on VCR at our hospital from March 2003 to March 2013.Results There were 19 boys and 14 girls.Their age during an initial diagnosis of KHE was 1 day to 10 months.Seven lesions were located in superficial soft tissue while deep soft tissue was involved in 26 cases.All cases were diagnosed with thrombocytopenia and 25 had coagulopathy.None of them was responsive to steroids before starting VCR treatment.And 22 cases achieved complete remission with a normalization of platelet count within (7.5 ± 5.2) weeks after VCR treatment.The masses shrank in size or became rofter at an average of (4.9 ± 2.6) weeks.Two cases had a partial response and 1 case stayed in treatment.And 8 cases had no obvious response to VCR and switched to other treatments.For 24 withdrawal cases,the average course of treatment was (31.1 ± 6.0) weeks.The mean and longest follow-up periods was 3.7 and 7.1 years respectively.And there was no recurrence.Conclusions VCR is both safe and effective in the treatment of steroid-resistant cases of KHE with KMP.And it should be app1ied early and recommended as a first line option.