石骨症骨髓活检的临床病理研究  

Clinical pathogenesis of in vivo detection of marrow for diagnosis of osteopetrosis

作　　者：刘芳[1] 顾莹莹[1] 何萍[1] 刘慕嫦[1] 林云恩[1] 莫明聪[1] 

机构地区：[1]广州医学院第一附属医院病理科,广东广州510120

出　　处：《中国热带医学》2006年第8期1441-1443,共3页China Tropical Medicine

摘　　要：目的探讨石骨症的骨髓活检的临床病理特点。方法常规HE染色,特殊染色氢氧化银氨液浸染法。结果骨髓活检示骨小梁增宽,密集,相互弯曲连接成网状或编织样,有钙盐沉着及不规则钙盐沉着线,无板层结构,骨髓腔严重狭窄,少量纤维组织增生,骨内膜细胞亦增生,仅见少量可识别的成熟粒系和红系细胞,未见巨核细胞,可见血窦扩张。结论石骨症是罕见的家族遗传性疾病,临床缺乏特征,患儿发育迟缓,肝脾大,贫血,骨髓活检显示骨髓腔严重狭窄,骨小梁有钙盐沉着,造血细胞显著减少等特点。Objective To discuss the clinical and pathological features of bone marrow biopsy of osteopetrosis. Methods Routine H - E staining and Argento - Ammonia hydroxide staining was carried out. Results by bone marrow biopsy, It was observed that the bone trabeculas was enlarged, concentrated and bended each other, looking like reticular patterns or knitting! calcium salts deposited in bone trabeculas, there was irregular line with calcium salts deposit without layer - structure in bone trabeculas. Medullary cavity of bone was constrictive seriously and a little fibrous tissue hyperplasia. Indosteum cell hyperplasia was also noticed ; there only a little mature granulocytic cell and erythrocyte identified and no megnkaryocyte could be observed; sinusoid was dilatant. Conclusion Osteopetrosis is a rare kind of disease which belongs to kindred genetic disease without typical clinical features, The main symptoms in the chidlren is growing slowly, hepatosplenomegaly, anemia and bone marrow biopsy displays the following characteristics such as medullary cavity of bone constrictive seriously, calcium salts deposit in bone trabeculas and significant decrease of hematopoiesis.

关 键 词：石骨症 骨髓活检 临床病理特点 

分 类 号：R681[医药卫生—骨科学]