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目的提高对儿童 Kasabach-Merritt 综合征(KMS)的临床及其 CT 和(或)MRI 表现的认识。方法分析总结6例 KMS 患儿的临床表现及其 CT 和(或)MRI 特点。结果 6例患儿均有血小板减少等消耗性凝血功能异常。3例 MRI 分别显示后纵隔脊柱旁、右侧心包及左上臂皮肤及皮下软组织 T_1WI 等、低信号,T_2WI 等、高信号病灶,增强呈不均匀显著强化;1例脾脏异常增大,CT 平扫为均匀稍低密度,增强呈弥漫性不均匀强化,MR T_1WI 呈低信号,T_2WI 呈高信号,增强后呈不均匀显著强化;1例除多根长骨干骺端骨质稀疏、骨皮质变薄外,脾脏亦增大,CT 平扫见多个大小不等低密度灶,增强早期见病灶周边强化,延迟相部分病灶有对比剂逐渐充填;1例肝左叶巨大低密度肿块,增强早期病灶周边强化,延迟相部分病灶见对比剂逐渐充填。结论血小板减少及凝血功能障碍提示可能合并大面积血管瘤;脾脏增大伴密度或 MRI 信号弥漫、局灶性降低应考虑血管瘤可能。
Objective To improve the clinical and CT and / or MRI findings of children with Kasabach-Merritt syndrome (KMS). Methods The clinical manifestations, CT and / or MRI features of 6 KMS children were analyzed and summarized. Results 6 cases of children with thrombocytopenia and other consumptive coagulation dysfunction. Three cases of MRI showed T_1WI and other soft tissue around the posterior mediastinum, the right pericardium, the left upper arm skin and subcutaneous soft tissue, low signal, T_2WI and other high signal lesions were significantly enhanced uneven enhancement; 1 case of abnormal spleen increased CT scan For a slightly lower density, enhanced diffuse heterogeneous enhancement, MR T_1WI showed low signal, T_2WI showed high signal enhancement was uneven significantly enhanced; in addition to a number of long bone metaphysis bone sparse, cortical thinning In addition, splenic enlargement was also seen in CT scan. A variety of low-density lesions ranging from CT to CT were seen in the CT scan. The peritumoral enhancement of early lesions was enhanced, and some of the lesions in the delayed phase were gradually filled with contrast media. One case of low- Peripheral enhancement, delayed phase contrast agent see some of the lesions gradually filled. Conclusions Thrombocytopenia and coagulation dysfunction suggest that large hemangiomas may be combined. The splenomegaly may be accompanied with density or MRI signal diffuseness. The focal reduction should be considered for possible hemangiomas.