RICH新生儿期出现血小板减少和凝血障碍一例报告并文献回顾  

Neonatal RICH associated with thrombocytopenia and coagulopathy： a case report and literature review

作　　者：高庆文[1] 沈卫民[1] 严俊[1] 陈海妮[1] 季易[1] 邹继军[1] 陈建兵[1] 崔杰[1] Gao Qingwen;Shen Weimin;Yaa Jun;Chen Haini;Ji Yi;Zou Jijun;Chen Jianbing;Cui Jie(Department of Burn-Plastic Surgery, Children＇s Hospital of Nanjing Medical University, Nanjing 210008, China)

机构地区：[1]南京医科大学附属儿童医院烧伤整形科,210008

出　　处：《中华整形外科杂志》2018年第6期488-493,共6页Chinese Journal of Plastic Surgery

摘　　要：2015年3月收治1例快速消退型先天性血管瘤（rapidly involuting congenital hemangioma, RICH）伴血小板减少（thrombocytopenia, TP）和凝血病（coagulopathy, CP）的新生儿，先后采用血小板输注和栓塞治疗（无水乙醇、聚桂醇）后，患儿血小板及凝血功能很快恢复正常，瘤体明显地消退萎缩，随后行手术切除。患儿在输注1次血小板后其PLT即恢复正常，多次复查凝血功能亦正常。瘤体明显变平、塌陷和萎缩，表面血管影减少、变淡。术后切口愈合良好，无复发，左上肢功能无受限。RICH是一种较少见的脉管肿瘤，临床上可出现TP和CP的并发症，这与新生儿期的Kaposi型血管内皮瘤伴Kasabach-Merritt现象非常类似，由于这两者的治疗方案及预后均有显著差异，需要仔细鉴别。低流量的静脉畸形或静脉淋巴管畸形也可出现消耗性凝血障碍和轻度的血小板减少。We treated a neonate who was diagnosed as rapidly involuting congenital hemangioma （RICH）. With a review on the clinical manifestation, differential diagnosis, and treatment of RICH that associated with thrombocytopenia（TP）and coagulopathy（CP）and received platelet transfusions and embolization therapy including absolute ethanol and polidocanol in our hospital in March of 2015. The platelets and coagulation function soon returned to normal, the tumor involuted significantly. Surgical excision was proceeded subsequently. The platelets returned to normal level after a one-time platelet transfusion, meanwhile, multiple reexaminations of blood coagulation function were normal. Postoperatively, incision wound healed well. There was no recurrence, and the functional recovery of upper limb was satisfactory. RICH is a rare type of vascular tumor which may present with TP and CP similar to KHE-KMP in the neonatal period. More attention should be addressed to identify these two diseases, because the treatment and prognosis of which have significant differences. Consumption of coagulation factors with milder platelet decrease may also be a complication of slow flow venous or venolymphatic malformations.

关 键 词：快速消退型先天性血管瘤 血小板减少 凝血病 卡梅现象 

分 类 号：R722.1[医药卫生—儿科]