Proteus综合征并发脾脏巨大血管瘤与慢性DIC——两例报告及文献复习  

Proteus syndrome with a giant hemangiomas in the spleen associated with chronic DIC two case report and literature review

作　　者：王兆钺[1] 苏雁华[1] 杨海燕[1] 余自强[1] 曹丽娟[1] 赵小娟[1] 胡浩[2] 詹升华[3] 阮长耿[1] 

机构地区：[1]苏州大学附属第一医院血液科、江苏省血液研究所,215006 [2]苏州大学附属第一医院普外科,215006 [3]苏州大学附属第一医院病理科,215006

出　　处：《中华血液学杂志》2007年第3期152-155,共4页Chinese Journal of Hematology

基　　金：江苏省卫生厅重点科技项目（K200401）

摘　　要：目的报道2例Proteus综合征并发脾脏巨大血管瘤与慢性DIC患者的临床、实验室止血检查与病理的特点及其治疗。方法用彩色B超与MRI对血管瘤做显像检查，分析Proteus综合征的脾脏巨大血管瘤的病理特征，检测患者的实验室止血指标。结果首次报道了2例并发脾脏巨大血管瘤的Proteus综合征患者，并导致慢性DIC（Kasabach-Merritt综合征），患者在经脾切除后治愈。结论Proteus综合征并发巨大血管瘤时可引起慢性DIC。患者的血浆纤维蛋白原水平显著降低有助于与其他病因的DIC的鉴别。Objective To investigate the clinical manifestations, pathologic features and laboratory findings in two Proteus syndrome patients with giant hemangiomas in the spleen and chronic DIC. Methods Ultrasound imaging and magnetic resonance imaging （ MRI ） were used for analysing the characteristics of the giant hemangiomas in the spleen. The spleen specimen was examined pathologically for the feature of the hemangioma. Homostatie tests were performed by routine laboratory methods. Results Two Proteus syndrome patienta with giant hemangiomas in the spleen causing chronic DIC （ Kasabach-Merritt syndrome） were first reported. They were recovered after spleneetomy. Conclusion Proteus syndrome when accompanied giant hemangioma could cause chronic DIC. Significantly decreased plasma fibrinogen level in this ease might be helpful for the differential diagnosis from DIC caused by other diseases.

关 键 词：PROTEUS综合征 弥散性血管内凝血 血管瘤 脾脏 

分 类 号：R686[医药卫生—骨科学]