摘要
目的探讨血友病性假肿瘤的影像学表现及相关临床特点,增加对罕见出血性疾病的影像诊断及临床处理思路。方法回顾性分析15例临床或病理确认的血友病性假肿瘤,结合其分型分级、病史、外伤史及伴随症状,总结其发生部位、大小、骨质改变、软组织等多种影像学特征,并进行统计分析。结果共完成血友病A型病例15例,病史1~35年,临床表现起初症状轻微,后发觉进行性肿块伴肿胀疼痛。其中行DR 6例,CT 9例,MRI 5例,病变最大径3.0~24.7 cm,平均(8.4±6.1)cm。病变位于股骨(5/15)、胫骨(1/15)、髂骨(3/15)、耻骨(2/15)、膝关节(3/15)、肘关节(1/15)。骨质改变呈膨胀性6例,溶骨性3例,囊样3例,受压迫变薄1例;骨质边缘硬化3例,伴病理性骨折3例,伴软组织肿块11例。结论血友病性假肿瘤的影像学表现具有一定特征。由于其较罕见且易误诊,凝血功能障碍等多重挑战,突出了术前全面影像学检查及确诊的重要意义。
Objective To investigate the imaging manifestations and related clinical features of hemophilia pseudotumor,and to increase the imaging diagnosis and clinical management of rare hemorrhagic diseases.Methods 15 cases of hemophilia pseudotumor confirmed by clinical or pathological diagnosis were retrospectively analyzed.Combined with their classification,medical history,history of trauma and accompanying symptoms,the imaging features such as location,size,bone changes and soft tissue were summarized and statistically analyzed.Results A total of 15 cases of hemophilia A were completed,with A history of 1-35 years.The clinical manifestations were mild at first,and then progressive mass with swelling and pain was found.The X-ray,CT and MRI examinations were performed in 6,9 and 5 cases respectively.The size of pseudo-tumor was 3.0-24.7 cm,mean(8.4±6.1)cm.In 15 cases of hemophilic pseudotumor,there were 5 cases in femur,1 case in tibia,3 cases in ilium,2 cases in pubis,3 cases in the knee-joint and 1 case in the elbow-joint.The images demonstrated expansive bone destruction(n=6),expansive and lytic bone destruction(n=3),cystic bone destruction(n=3),erosive bone destruction(n=1).Bone destruction with edge sclerosis(n=3),bone destruction with pathologic fracture(n=3),and soft tissue mass(n=11).Conclusion The imaging findings of hemophilia pseudotumor have certain characteristics.Due to its rarity,easy misdiagnosis and multiple challenges such as coagulation dysfunction,it highlights the importance of preoperative comprehensive imaging examination and diagnosis.
作者
汪纯洁
翁春娇
WANG Chun-jie;WENG Chun-jiao(Hangzhou No.1 People's Hospital,Hangzhou,Zhejiang,310006,China;First Affiliated Hospital of Soochow University,Suzhou,Jiangsu,215006,China)
出处
《中国血液流变学杂志》
CAS
2022年第3期463-470,F0003,共9页
Chinese Journal of Hemorheology
关键词
血友病性假肿瘤
影像学表现
X线计算机
磁共振成像
hemophilic pseudotumor
imaging findings
X ray computer
magnetic resonance imaging
