16例新生儿卡梅综合征临床分析

Clinical features of Kasabach-Merritt syndrome:an analysis of 16 neonates

目的分析总结新生儿卡梅综合征(Kasabach-Merritt syndrome,KMS)的临床特点、治疗及预后,为优化该病的诊断及治疗提供参考依据。方法回顾性分析安徽医科大学附属省儿童医院2016年1月至2020年12月收治的16例KMS新生儿的住院及随访资料。结果 16例KMS患儿中,男13例(81%),女3例(19%),入院年龄为1 h至10 d。13例(81%)为皮肤血管瘤(头面部2例、躯干5例、四肢6例),3例(19%)为肝脏血管瘤。10例(62%)临床表现以出血倾向、全身散在瘀点瘀斑为主。16例患儿均有不同程度的血小板减少及凝血功能异常。所有患儿入院后均采取糖皮质激素保守治疗,7例(44%)有效,其中复发4例。糖皮质激素治疗无效患儿中3例接受西罗莫司治疗,1例治疗4周后瘤体缩小58.8%,血小板计数及凝血功能恢复正常;2例瘤体无明显缩小,血小板计数无明显回升,联合博来霉素动脉栓塞治疗4周后瘤体缩小(43.7±0.4)%,血小板计数及凝血功能恢复正常。4例单独接受博来霉素动脉栓塞治疗4周后瘤体缩小(52.0±3.4)%,血小板计数及凝血功能恢复正常。2例接受外科钝性+锐性剥离切除术,术中切除全部瘤体,术后无感染及复发,血小板计数及凝血功能恢复正常,1例术后病理结果为卡波西样血管内皮瘤。结论 KMS具有特征性临床表现、组织病理特征及实验室检查结果。对激素反应不敏感的KMS通过动脉栓塞和西罗莫司治疗可取得较好的疗效。

Objective To study the clinical features,treatment,and prognosis of neonates with Kasabach-Merritt syndrome(KMS),and to provide a reference for optimizing the diagnosis and treatment of this disease.Methods A retrospective analysis was performed for the clinical and follow-up data of 16 neonates with KMS who were admitted to the Anhui Children’s Hospital,Anhui Medical University,from January 2016 to December 2020.Results Of the 16 neonates,there were 13 boys(81%)and 3 girls(19%),with an age of 1 hour to 10 days on admission.Among these neonates,13(81%)had cutaneous hemangioma(2 in the head and face,5 in the trunk,and 6 in the extremities)and 3(19%)had liver hemangioma.The main clinical manifestations of bleeding tendency and scattered petechiae and ecchymosis were observed in 10 neonates(62%).All the 16 neonates had varying degrees of thrombocytopenia and coagulation disorders.They all received glucocorticoid treatment after admission and 7(44%)of them had response,among whom 4 experienced recurrence.Among the neonates with no response to glucocorticoid treatment,3 received sirolimus treatment,among whom 1 had the tumor volume reduced by 58.8%after 4 weeks of treatment,with platelet count and coagulation function returning to normal,while 2 had no significant reduction in tumor volume or significant increase in platelet count and achieved a tumor volume reduced by(43.7±0.4)%after 4 weeks of combined treatment with bleomycin arterial embolization,with platelet count and coagulation function returning to normal.After 4 weeks of bleomycin arterial embolization alone for 4 neonates,tumor volume was reduced by(52.0±3.4)%,and platelet count and coagulation function returned to normal.Blunt and sharp dissection was performed for 2 neonates.The tumor was removed completely during surgery in the 2 neonates,with no infection or recurrence after surgery,and platelet count and coagulation function returned to normal.The postoperative pathological examination showed Kaposiform hemangioendothelioma in 1 out of the 2 neonates.Conclusions KMS has characteristic clinical manifestations,histopathological features,and laboratory examination results.The KMS neonates who are not sensitive to glucocorticoids can achieve a good curative effect through arterial embolization and sirolimus treatment.