摘要
目的:探讨良、恶性石骨症的临床及X线平片的特征性表现,提高对本病的鉴别诊断水平。方法:对12例良恶性石骨症患者的临床资料及X线平片表现进行回顾性分析,其中良性5例,恶性7例。结果:良性病变的临床特点为早期无症状,发病轻,进展慢。恶性型临床特点为发病早,进展快,进行性贫血,血小板减少,肝脾及淋巴结肿大。实验室检查主要指标:红细胞、血红蛋白及血小板减少。12例石骨症的基本X线表现为全身骨质硬化,髓腔变窄。其中5例良性病变的特征性X线征象:椎体呈"夹心蛋糕"征5例,髂骨呈"同心圆"征5例,伴发病理性骨折2例;7例恶性病变的特征性X线征象:长、短骨呈"棒球杆"征7例,"骨中骨"征6例,肋骨呈"浆"征7例,头颅呈"面罩"征7例,椎体呈"夹心蛋糕"征2例,伴发佝偻病改变7例。结论:良恶性石骨症各具有一定的特征性X线表现,结合临床表现及实验室检查,X线检查对本病的诊断和鉴别诊断具有重要价值,对临床早期治疗有指导意义。
Objective：To discuss the clinical and radiographic characteristics of benign and malignant osteopetrosis,in order to improve the diagnosis accuracy of the disease entity.Methods：The clinical materials and radiographic appearances of 12 cases with benign osteopetrosis（BO,5 cases） and malignant osteopetrosis（MO,7 cases） were retrospectively analyzed.Results：No clinical signs and symptoms were revealed at early stage of BO,slow progression of the disease was seen.As for MO,early invasion,rapid progression accompanied by progressive anemia,thrombocytopenia and hepatomagaly,splenomagaly as well as lymphadenopathy were observed;the main abnormal laboratory findings were erythropenia,hypohemoglobinemia,and thrombocytopenia.The basic radiographic manifestations of osteopetrosis were osteosclerosis of all bones and narrowing of bone-marrow cavities.Of the 5 cases with BO,the radiographic findings included ＂sandwich sign＂ of vertebral body（5 cases）,＂concentric circle sign＂ of iliac bone（5 cases）,accompanied with pathologic fracture（2 cases）.As for the 7 cases with MO,＂baseball-bat sign＂ of long and short bones（7 cases）,＂bone within bone sign＂（6 cases）,and 7 are plasma-like ribs ＂mask-like sign＂ of skull（7 cases）,＂sandwich sign＂ of vertebral body（2 cases） and accompanied ＂rickets-like＂ changes（7 cases） were assessed.Conclusion：Characteristic radiographic manifestations could be seen in benign as well as malignant osteopetrosis,radiographic in combination with clinical and laboratory findings are helpful in the diagnosis,differential diagnosis and treatment guidance of the disease.
出处
《放射学实践》
北大核心
2011年第7期749-752,共4页
Radiologic Practice
关键词
石骨症
放射摄影术
骨骼病变
诊断
Osteopetrosis
X-ray manifestation
Radiography
Bone diseases
Diagnosis
