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婴儿期的卡萨巴赫-梅里特综合征。

Kasabach-Merritt syndrome in infants.

作者信息

Esterly N B

出版信息

J Am Acad Dermatol. 1983 Apr;8(4):504-13. doi: 10.1016/s0190-9622(83)70056-3.

Abstract

Kasabach-Merritt syndrome consists of thrombocytopenia, microangiopathic hemolytic anemia, and an acute or chronic consumption coagulopathy in association with a rapidly enlarging hemangioma. Although the potential for serious morbidity is great, the condition remits when the hemangioma begins to involute. Four infants with large congenital cavernous hemangiomas and Kasabach-Merritt syndrome are described. All four received oral prednisone for variable periods; two received aspirin and one dipyridamole. Two patients required intravenous heparin because of life-threatening disseminated intravascular coagulation and bleeding. Two patients experienced almost total involution of their hemangiomas by the age of 2 years, and one by the age of 13 months. The fourth patient showed signs of beginning resolution of the hemangioma at the age of 8 months and hematologic values returned to normal levels. No side effects from therapy were noted in any of these infants.

摘要

卡萨巴赫-梅里特综合征包括血小板减少、微血管病性溶血性贫血以及与迅速增大的血管瘤相关的急性或慢性消耗性凝血病。尽管严重发病的可能性很大,但当血管瘤开始消退时,病情会缓解。本文描述了4例患有大型先天性海绵状血管瘤和卡萨巴赫-梅里特综合征的婴儿。所有4例均接受了不同疗程的口服泼尼松治疗;2例接受了阿司匹林治疗,1例接受了双嘧达莫治疗。2例患者因危及生命的弥散性血管内凝血和出血而需要静脉注射肝素。2例患者在2岁时血管瘤几乎完全消退,1例在13个月时消退。第4例患者在8个月时显示出血管瘤开始消退的迹象,血液学指标恢复到正常水平。这些婴儿均未出现治疗的副作用。

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