先天性红细胞生成性卟啉病的骨髓红系超微结构观察(附1例报告)  

The Clinical and Ultrastructural Observation of Congenital Erythropoietic Porphyria——with a case report

作　　者：张金梅[1] 冉家彦[1] 崔月娥 高秀花 王椿[1] 

机构地区：[1]山西医学院附属二院,030001

出　　处：《临床血液学杂志》1993年第3期109-111,共3页Journal of Clinical Hematology

摘　　要：本文对1例先天性红细胞生成性卟啉病的实验室及骨髓红系超微结构进行了系统观察。着重对其骨髓幼红细胞超微结构的异常变化及荧光显微镜观察的发现作了描述和讨论。A case of congenital erythropoietic porphyria was reported and re-seached in this paper. Its clinical features were photosensitive dermatitis, pink por-phyrinuria and hemolytic anemia. Regressive phenomenon and ferruginous granulesaccumulation were observed in mitochondria in some of basophilic normoblasts. Chro-matinic description was abnormal in erythroblastic nuclei. Large pieces of lower elec-tion density substances and inclusion bodies were abserved in erythroblastic nuclei.Yellow fluorescence was seen in nuclei and red fluorescence was aboserved in cellularplasma with fluorescence microscope in some of basophilicnormoblasts.

关 键 词：卟啉病 超微结构 荧光 

分 类 号：R559[医药卫生—血液循环系统疾病]