血友病B伴腹盆腔假肿瘤一例  

A Case of Hemophilia B with Abdominal and Pelvic Pseudotumor

作　　者：张婧 鞠满凯 张科民 陈玲玲 王玉华 张磊 杨仁池 薛峰 ZHANG Jing;JU Mankai;ZHANG Kemin;CHEN Lingling;WANG Yuhua;ZHANG Lei;YANG Renchi;XUE Feng(Thrombosis and Hemostasis Center,National Clinical Research Center for Blood Diseases,State Key Laboratory of Experimental Hematology,National Hemophilia Registry Center,Institute of Hematology&Blood Diseases Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Tianjin 300020,China;Department of Imaging,National Clinical Research Center for Blood Diseases,State Key Laboratory of Experimental Hematology,National Hemophilia Registry Center,Institute of Hematology&Blood Diseases Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Tianjin 300020,China;Coagulation Laboratory,National Clinical Research Center for Blood Diseases,State Key Laboratory of Experimental Hematology,National Hemophilia Registry Center,Institute of Hematology&Blood Diseases Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Tianjin 300020,China)

机构地区：[1]中国医学科学院血液病医院(中国医学科学院血液学研究所)、国家血液系统疾病临床医学研究中心、实验血液学国家重点实验室、国家血友病病例信息管理中心血栓止血诊疗中心,天津300020 [2]中国医学科学院血液病医院(中国医学科学院血液学研究所)、国家血液系统疾病临床医学研究中心、实验血液学国家重点实验室、国家血友病病例信息管理中心影像科,天津300020 [3]中国医学科学院血液病医院(中国医学科学院血液学研究所)、国家血液系统疾病临床医学研究中心、实验血液学国家重点实验室、国家血友病病例信息管理中心出凝血实验室,天津300020

出　　处：《罕见病研究》2022年第4期456-460,共5页Journal of Rare Diseases

摘　　要：血友病B是一种基因突变导致的凝血因子Ⅸ(FⅨ)缺乏性疾病,主要表现为关节、肌肉及深部组织出血。血友病假肿瘤是反复出血导致液化坏死的软组织形成的肿块,大部分假肿瘤发生在骨骼及肌肉处。本文报道了一例血友病B伴盆腹腔假肿瘤形成的患者,病变位置相对罕见,对患者进行积极有效的止血后,患者血尿症状逐渐好转。该病例提示血友病患者早期及时止血治疗是至关重要的。Hemophilia B is a genetic disorder caused by coagulation factor Ⅸ(FⅨ) deficiency, mainly manifesting as joint, muscle and deep tissue bleeding. Hemophilia pseudotumor is a mass formed by soft tissue liquefaction and necrosis caused by repeated bleeding. Most pseudotumors occur in the bone and muscle. We report a case of hemophilia B with pseudotumor formation in the pelvis and abdomen, where lesion location is relatively rare. After active and effective hemostasis, the patient’s hematuria symptom gradually improved. This case suggests that early and timely hemostatic treatment is crucial for patients with hemophilia.

关 键 词：血友病B 假肿瘤 影像学表现 

分 类 号：R445[医药卫生—影像医学与核医学] R554.1[医药卫生—诊断学]