摘要
目的对Erdheim-Chester病(Erdheim-Chester disease,ECD)的^(99m)Tc-亚甲基二膦酸盐(methylene diphosphonate,MDP)全身骨显像特点进行总结,以期为临床诊疗提供参考。方法本研究为描述性分析,研究对象为2015年1月—2022年12月北京协和医院^(99m)Tc-MDP全身骨显像示骨受累的ECD患者,总结病变累及部位、病变特征并分析骨骼病变评分与常见骨代谢分子标志物的相关性。结果共入选44例出现骨受累的ECD患者。^(99m)Tc-MDP全身骨显像示,全身骨骼均可受累,其中四肢骨(100%,44/44)、颅骨与颌面骨(84.1%,37/44)、肢带骨(65.9%,29/44)受累最为常见,肋骨与胸骨(38.6%,17/44)、脊柱(29.5%,13/44)受累相对少见。除累及部位具有特征性外,病灶呈双侧对称性分布是ECD患者^(99m)Tc-MDP全身骨显像的另一重要特征。其中以四肢骨、颌面骨、锁骨病变呈双侧对称性分布的患者比例较高,尤其下肢骨受累,病变均为双侧对称(100%)。四肢骨病变中,多为骨干和干骺端共同受累,81.8%(36/44)的患者下肢骨病变形成了以膝关节为中心的对称性干骺端和骨干高摄取表现;颌面骨病变主要表现为副鼻窦区域局灶性摄取增高,伴或不伴下颌骨弥漫性摄取增高,并可出现“林肯征”(61.1%,22/36)。Spearman相关性分析显示,骨骼病变评分与血钙、血磷、碱性磷酸酶、甲状旁腺激素等骨代谢分子标志物均无线性相关(P均>0.05)。结论ECD可出现全身多处骨骼受累,其中以四肢骨尤其下肢骨受累最为常见,并常伴颅骨和颌面骨病变;膝关节周围干骺端和骨干对称性摄取增高是^(99m)Tc-MDP骨显像最常见的特征,对ECD的诊断及鉴别诊断具有重要参考价值。
Objective To summarize the features of^(99m)Tc⁃methylene diphosphonate(MDP)whole⁃body bone scintigraphy(WBBS)in Erdheim⁃Chester disease(ECD)patients and to provide insights for clini⁃cal diagnosis and treatment.Methods Retrospective analysis was performed in ECD patients with bone lesion who underwent^(99m)Tc⁃MDP WBBS at Peking Union Medical College Hospital from January 2015 to December 2022.The bone involvement sites and characteristic features in^(99m)Tc⁃MDP WBBS were summarized,and the correlation between bone lesion scores and bone metabolic biomarkers was analyzed.Results A total of 44 ECD patients with bone involvement were included in the study.According to the^(99m)Tc⁃MDP WBBS,the most commonly affected sites were the bones of the extremities(100%,44/44),skull and maxillofacial bones(84.1%,37/44),and appendicular bones(65.9%,29/44),while involvement of the ribs and sternum(38.6%,17/44)and spine(29.5%,13/44)were less frequent.Bilateral and symmetric change of the le⁃sions was an important feature of^(99m)Tc⁃MDP WBBS in ECD.The proportion of patients with bilateral and sym⁃metric bone lesions in the extremities,maxillofacial area,and clavicle was relatively high,particularly in the lower extremities(100%).In extremity bone lesions,there was mostly joint involvement of diaphysis and me⁃taphysis,and 81.8%(36/44)patients demonstrated the typical scintigraphic characteristic:bilateral and sym⁃metrical increased uptake in the distal femur and proximal tibia.The maxillofacial lesions mainly presented with focal uptake increase in the region of the paranasal sinuses,with or without diffuse increased uptake in the man⁃dible⁃Lincoln sign(61.1%,22/36).Spearman correlation analysis revealed that the bone metabolic biomar⁃kers such as blood calcium,blood phosphate,alkaline phosphatase,and parathyroid hormone were not linearly correlated with bone lesion scores(all P>0.05).Conclusions ECD can cause multiple bone lesions,with in⁃volvement typically observed in the extremities,particularly in the lower extremities.Lesions in the skull and maxillofacial area are also frequently observed.Bilateral and symmetric uptake increase in the distal femur and proximal tibia is the most pathognomonic manifestation of^(99m)Tc⁃MDP WBBS,providing valuable information for diagnosing and differentiating ECD.
作者
马艳茹
牛娜
曹欣欣
李轶
景红丽
刘宇
陈黎波
MA Yanru;NIU Na;CAO Xinxin;LI Yi;JING Hongli;LIU Yu;CHEN Libo(Department of Nuclear Medicine,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;Department of Hematology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China)
出处
《协和医学杂志》
CSCD
2023年第4期781-786,共6页
Medical Journal of Peking Union Medical College Hospital
基金
中国医学科学院医学与健康科技创新工程(2022-I2M-JB-001)。
