综合序贯疗法治疗Kasabach-Merritt综合征

目的 分析Kasabach-Merritt综合征(KMS)的治疗方法。方法 对2005-2009年采用综合序贯疗法治疗的51例KMS患儿的临床和随访资料进行回顾性分析。其中男27例,女24例;年龄2d至2.5岁,中位年龄1.8个月。病变部位：颌面部20例,躯干4例,上肢11例,下肢16例。结果 51例KMS患儿中,3例（右颌面、左前臂、右大腿部各1例）因血管瘤意外破溃行急诊血管瘤切除手术,全部痊愈;余48例首先接受激素联合尿素局部注射治疗,其中激素敏感12例,有效15例,无效21例;对激素无效的21例患儿序贯给予丙种球蛋白治疗,敏感5例,有效7例,无效9例。单纯药物加局部尿素治疗共14例,11例痊愈,3例有效;血小板计数4～7 d恢复到正常的4例,8～14 d恢复的5例,15～28 d恢复的2例,超过28 d恢复的3例;瘤体6～ 12个月消失的6例,13～24个月消失的8例。对颌面部血管瘤行颈外动脉结扎置管介入治疗,共19例,全部痊愈;血小板计数术后4～7d恢复到正常的13例,8～14 d恢复的6例;瘤体6～ 12个月消失的12例,13～24个月消失的7例。药物治疗反应无效或病情反复的躯干、四肢血管瘤行择期血管瘤切除手术,共15例,痊愈14例,死亡1例。所有血管瘤切除手术中,行病变完全切除者10例,术后1～3d血小板恢复正常;病变大部切除者8例,术后8～ 14 d 血小板恢复正常。结论 综合序贯疗法治疗KMS效果确切,治愈率高,无明显不良反应。

Combined and sequential therapy for Kasabach-Merritt syndrome

ObjectiveTo review and summarize the clinical characteristics and therapeutic approaches of Kasabach-Merritt syndrome ( KMS). MethodsThe combined and sequential therapy was used to for 51 KMS infants between 2005 and 2009. And all cases were retrospectively analyzed. There were 27 males and 24 females with a median age of 1. 8 months ( range: 2 days to 2. 5 years).Among them,lesions were located in maxillofacial ( n = 20), truck ( n = 4), upper extremities ( n = 11 ) and lower extremities (n = 16). ResultsAmong them, 47 of 51 patients were cured, 3 cases were effectively treated and only 1 patient died of multiple organ failure post-operation.Of the 51 cases, emergency surgery was given in 3 cases while other 48 cases were initially treated with methylprednisolone. The responses were varied : excellent and rapid improvement ( n = 12 ) ; moderate response ( n = 15 ) and total failure ( n = 21 ).Immunoglobulin was used a second-line therapy for those (n = 21 ) unresponsive to steroid, with sensitive response( n = 5 ), effective response ( n = 7 ) and invalid response ( n = 9 ) respectively.And adjunct carbonyldiamide therapy was combined. Conservative treatment was tried in 14 patients. Then 11 curative cases and 3 effective cases were observed. The thrombocyte count in 4 cases returned to normal within 4 -7days, 5 cases within 8 - 14 days, and 2 cases within 15 -28 days. In the remaining 3 cases, it normalized more than 28 days.The hemangiomas in 6 cases disappeared completely in 6 - 12 months and 8 cases disappeared in 13 - 24 months.19 patients were treated with the ligation of external carotid artery, the insertion of a tube inside the lesion and the injections of carbonyldiamide and methylprednisolone. The platelet counts of 13 cases increased to normal in 4 -7 days of the treatment, and 6 cases in 8 - 14 days.The tumors of 12 cases disappeared within 6 - 12 months, and 7 cases within 13 - 24 months. Combined surgical resection including emergency operation (n = 3 ) and selective operation (n = 15 ) was performed in 18 patients. And all cases except for one were cured. The platelet counts of that the hemangiomas of 10cases were excised surgically completely increased rapidly and reached the normal range in post-operative 1 - 3 days. The counts of 8 cases after subtotal resection increased to normal within 8 - 14 days. Conclusion A stepwise multimodal approach is recommended for the treatment of KMS.With a higher curative rate, it has minimal side effects.