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

摘要:

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。

Abstract:

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.