Haahr V, Jacobsen E, Bendix K, Nielsen J L, Peterslund N A
Medicinsk-haematologisk afdeling, Arhus Amtssygehus.
Ugeskr Laeger. 1994 Oct 10;156(41):6011-4.
Kasabach Merritt syndrome, first recognized in 1940, is characterized by haemangiomatosis, thrombocytopenia and intravascular coagulation. It is most often seen in children, rarely in adults. The mortality rate is 20-30%. Treatment is by removing the haemangiomatosis and correcting the consumptive coagulopathy. The purpose of this paper is to present a patient with Kasabach Merritt syndrome with haemangiomatosis in the spleen and the liver. A review of the relevant literature is given.
卡萨巴赫-梅里特综合征于1940年首次被发现,其特征为血管瘤病、血小板减少症和血管内凝血。该病多见于儿童,成人罕见。死亡率为20% - 30%。治疗方法是切除血管瘤病并纠正消耗性凝血病。本文旨在介绍一名患有脾脏和肝脏血管瘤病的卡萨巴赫-梅里特综合征患者,并对相关文献进行综述。