新生儿Kasabach-Merritt综合征1例并文献复习
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摘要
患儿男,生后1天,出生即发现右侧大腿有一巨大暗红色包块,实验室检查提示血小板减少、低纤维蛋白原血症,彩超提示血管性病变,诊断为Kasabach-Merritt综合征。急性期静脉应用糖皮质激素、长春新碱,后期辅以静滴、局部注射干扰素的序贯内科治疗,治疗2周后血小板及凝血功能恢复正常,总疗程1年4个月,瘤体全部消失,目前遗留皮肤松弛、色素沉着。
A newborn male infant of one-day old was born with a relatively big dark red mass in his right thigh. Laboratory test suggesed thrombocytopenia and hypofibrinogenemia,which vascular anomalies was seen by color Doppler USG. It was diagnosed as Kasabach-Merritt syndrome. During acute stage,intravenous injection of glucocorticoids and vincristine was recommended. In the later stage,intravenous injection and local injection of interferon in sequential treatment were used as the maintenance treatment. After two weeks of treatment,the platelet count and coagulation function returned to normal. After 1 year and 4 months treatment,the tumor was completely cured with no relapse. However,the skin laxity and pigmentation still remained.
A newborn male infant of one-day old was born with a relatively big dark red mass in his right thigh. Laboratory test suggesed thrombocytopenia and hypofibrinogenemia,which vascular anomalies was seen by color Doppler USG. It was diagnosed as Kasabach-Merritt syndrome. During acute stage,intravenous injection of glucocorticoids and vincristine was recommended. In the later stage,intravenous injection and local injection of interferon in sequential treatment were used as the maintenance treatment. After two weeks of treatment,the platelet count and coagulation function returned to normal. After 1 year and 4 months treatment,the tumor was completely cured with no relapse. However,the skin laxity and pigmentation still remained.
引文
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[8]Croteau SE,Gupta D.The clinical spectrum of kaposiform hemangioendothelioma and tufted angioma[J].Semin Cutan Med Surg,2016,35(3):147-152.
[9]Yoon HS,Lee JH,Moon HN,et al.Successful treatment of retroperitoneal infantile hemangioendothelioma with Kasabach-Merritt syndrome using steroid,alpha-interferon,and vincristine[J].Journal of Pediatric Hematology/Oncology,2009,31(12):952.
[10]Chiu YE,Drolet BA,Blei F,et al.Variable response to propranolol treatment of kaposiform hemangioendothelioma,tufted angioma,and Kasabach-Merritt phenomenon[J].Pediatr Blood Cancer,2012,59(5):934-938.
[11]陈运洪,杨丽华,王斌,等.α-2b干扰素成功治疗婴儿巨大血管瘤5例报告[J].临床儿科杂志,2011,29(6):576-579.
[12]Schroeder U,Lauten M,Stichtenoth G,et al.Laryngomalacia and complicated,life-threatening m TOR-positive Kaposiform hemangioendothelioma cured by Supraglottoplasty and sirolimus[J].Klin Padiatr,2014,226(6-7):362-368.
[13]Adams DM,Trenor CR,Hammill AM,et al.Efficacy and safety of sirolimus in the treatment of complicated vascular anomalies[J].Pediatrics,2016,137(2):e20153257.
[14]O'Rafferty C,O'Regan GM,Irvine AD,et al.Recent advances in the pathobiology and management of Kasabach-Merritt phenomenon[J].Br J Haematol,2015,171(1):38-51.
[2]Kwok-Williams M,Perez Z,Squire R,et al.Radiotherapy for life-threatening mediastinal hemangioma with KasabachMerritt syndrome[J].Pediatr Blood Cancer,2007,49(5):739-744.
[3]师晓东,张朝霞,刘嵘,等.婴儿血管瘤合并KasabachMerritt综合征1例报告并文献复习[J].中国医刊,2016,51(8):83-86.
[4]郭新奎,李鹏,郭正团,等.Kasabach-Merritt综合征38例回顾性分析[J].中国皮肤性病学杂志,2011,25(10):775-777.
[5]燕丽,徐哲,孙玉娟,等.婴儿Kasabach-Merritt综合征16例临床回顾性分析[J].实用皮肤病学杂志,2015,8(4):257-259.
[6]廖清奎.小儿血液病基础与临床[M].北京:人民卫生出版社,2001:702-703.
[7]董长宪,雷红召,黄静,等.药物联合手术治疗KasabachMerritt综合征[J].中华整形外科杂志,2013,29(2):104-108.
[8]Croteau SE,Gupta D.The clinical spectrum of kaposiform hemangioendothelioma and tufted angioma[J].Semin Cutan Med Surg,2016,35(3):147-152.
[9]Yoon HS,Lee JH,Moon HN,et al.Successful treatment of retroperitoneal infantile hemangioendothelioma with Kasabach-Merritt syndrome using steroid,alpha-interferon,and vincristine[J].Journal of Pediatric Hematology/Oncology,2009,31(12):952.
[10]Chiu YE,Drolet BA,Blei F,et al.Variable response to propranolol treatment of kaposiform hemangioendothelioma,tufted angioma,and Kasabach-Merritt phenomenon[J].Pediatr Blood Cancer,2012,59(5):934-938.
[11]陈运洪,杨丽华,王斌,等.α-2b干扰素成功治疗婴儿巨大血管瘤5例报告[J].临床儿科杂志,2011,29(6):576-579.
[12]Schroeder U,Lauten M,Stichtenoth G,et al.Laryngomalacia and complicated,life-threatening m TOR-positive Kaposiform hemangioendothelioma cured by Supraglottoplasty and sirolimus[J].Klin Padiatr,2014,226(6-7):362-368.
[13]Adams DM,Trenor CR,Hammill AM,et al.Efficacy and safety of sirolimus in the treatment of complicated vascular anomalies[J].Pediatrics,2016,137(2):e20153257.
[14]O'Rafferty C,O'Regan GM,Irvine AD,et al.Recent advances in the pathobiology and management of Kasabach-Merritt phenomenon[J].Br J Haematol,2015,171(1):38-51.