卡梅综合征研究现状及进展

Research progress of Kasabach-Merritt syndrome

卡梅综合征又称血小板减少性紫癜的毛细血管瘤综合征,以严重的血小板减少和低纤维蛋白原血症为特征,与罕见的血管肿瘤相关,如卡波西样血管内皮瘤,部分与丛生血管瘤具有相关性,以血小板减少、纤维蛋白原降低、纤维蛋白分裂产物增多和肿瘤快速生长为特征,严重时可发生贫血,危及生命,因此及时诊断和治疗很重要。我们对卡梅综合征的组织免疫学特点、病理生理发病机制、临床表现、诊断、治疗和预后展望的研究进展进行综述。

Kasabach-Merritt syndrome( KMS),also known as thrombocytopenic purpura,is characterized by severe thrombocytopenia and hypoproteinemia. It is associated with rare vascular tumors,such as Kaposi like hemangioendothelioma and with hemangioma conglomeration. It is characterized by thrombocytopenia,decreased fibrinogen,increased mitotic products of fibrin and rapid growth of tumor. In severe cases,it may cause anemia and life-threatening. In case of timely diagnosis and treatment,the patients can be saved. In order to assist clinicians in diagnosis and treatment in time,this review focuses on the histological and immunological characteristics,pathophysiological pathogenesis,clinical manifestations,diagnosis,treatment and prognosis of KMS.