About I, Capdeville J, Bernard P, Lazorthes F, Boneu B
Service de médecine interne A, hôpital de Foix, France.
Rev Med Interne. 1994;15(12):846-50. doi: 10.1016/s0248-8663(05)82844-9.
The Kasabach-Merritt syndrome was first described in children with cutaneous hemangiomas, but it can exceptionally be associated with visceral hemangiomas, especially in adults. Clotting and fibrinolysis within the hemangioma are thought to cause the coagulopathy observed in the so-called Kasabach-Merritt syndrome. This localised form of intra-vascular coagulation can progress to a secondary increased systemic fibrinolysis with fatal outcome for 20 to 30% of the patients. A transient control of hematologic abnormalities can frequently be obtained with blood product support (platelets, fibrinogen, fresh plasma, cryoprecipitates) and heparinotherapy. But in the adult, the only radical alternative is surgical excision if technically feasible. We reported here the case of a 43 year-old woman with a giant unresectable hepatic hemangioma complicated with a Kasabach-Merritt syndrome.
卡萨巴赫-梅里特综合征最初在患有皮肤血管瘤的儿童中被描述，但也可能罕见地与内脏血管瘤相关，尤其是在成人中。血管瘤内的凝血和纤溶被认为是导致在所谓的卡萨巴赫-梅里特综合征中观察到的凝血病的原因。这种局部形式的血管内凝血可进展为继发性全身性纤溶增加，20%至30%的患者会因此死亡。通过血液制品支持（血小板、纤维蛋白原、新鲜血浆、冷沉淀）和肝素治疗，常可实现对血液学异常的短暂控制。但在成人中，如果技术上可行，唯一的根治方法是手术切除。我们在此报告了一例43岁女性患者，患有巨大的不可切除的肝血管瘤并伴有卡萨巴赫-梅里特综合征。
