进行性骨干发育不全临床病理观察  

Progressive diaphyseal dysplasia:a clinical and pathologic analysis

作　　者：闫利娟 张龙[2] 顾盼[2] 朱旭友[2] 方霞[2] 张育东 顾俊[2] 郑张军 

机构地区：[1]洛阳市第二中医院病理科,河南洛阳471003 [2]同济大学附属同济医院,上海200065

出　　处：《诊断病理学杂志》2015年第4期217-220,共4页Chinese Journal of Diagnostic Pathology

基　　金：上海市科学技术委员会科研基金资助项目(034119868);上海市科学技术委员会医学重点科研基金资助项目(09411951600)

摘　　要：目的探讨进行性骨干发育不全(PDD)的临床病理特征,以提高对该病的诊断水平。方法对1例进行性骨干发育不全的临床表现、影像学特点、病理形态特征进行分析,并结合文献进行讨论。结果患者为青年女性,主要临床表现为四肢变粗伴下肢水肿、疼痛。X线平片显示多发骨改变。病理形态示骨细胞增生明显活跃,可见大量的骨母细胞,骨内、外膜均增生,骨小梁增粗、肥大,排列紊乱。结论 PDD的临床和病理缺乏特征性,明确诊断依赖于临床、影像学及病理学的结合。Objective To investigate the clinicopathologic characteristics of progressive diaphyseal dysplasia （PDD） for improving the accurate diagnosis of it. Methods A case of PDD was analyzed on its clinical symptoms, imaging features, pathological characteristics and related literature was reviewed. Results The patient, 26 year-old female, complained with thicker limbs, oedema legs and pain of limbs. The X-ray showed the change of multiple bones. Pathological morphology showed active hyperplasia of osteocytes, with a lot of osteoclasts. There were hyperplasia of endosteum and periosteum, thick and flat bone trabeculae with disorder arrangements. Conclusion The clinical and pathological manifestation in PDD is not specific. The correct diagnosis depends on the analysis of clinical, radiological and pathological features.

关 键 词：进行性骨干发育不全 临床病理 诊断 

分 类 号：R738.1[医药卫生—肿瘤]