以"凝血异常"为首发症状的卡-梅综合征三例及文献复习

Objective: To study the clinical feature，diagnosis, treatment of Kasabach-Merritt Syndrome and improve the understanding of the disease. Methods: A retrospective summary about 3 cases of Kasabach-Merritt Syndrome were analyzed from November 2007 to April 2013.Combined with literature review. Results: 2 cases of girls and 1 case was boy， age was 3, 4years old, 2month separately. 3 cases were shown as erythema of the skin and joint or ecchymosis, 1 case of splenomegaly. 3 cases were characterized by different degrees of coagulopathy. 1 case prothrombin time (PT), activated partial thromboplastin time (APTT) ，thrombin time (TT), fibrinogen (Fg) are not solidification, antithrombin Ⅲ increased. Blood routine examination: 2 cases with decreased hemoglobin (Hb), 1 case of simple Plt decrease. 2 cases have been done hemangioma vein embolization and spleen resection respectively. The diseases were remission, routine blood and the blood coagulation returned to normal. 1 case died of severe bleeding. Conclusions: Kasabach-Merritt Syndrome is a rare infant and young children period with thrombocytopenic purpura and a coagulopathy hemangioma lesion. Clinicians should improve the understanding of disease and reduce misdiagnosis by combining the clinical symptoms and blood routine, blood coagulation, imaging examination and histopathologic examination.