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[黏多糖贮积症 VI 型中的阿尔德异常。细胞学、细胞化学及超微结构研究]

[Alder's anomaly in mucopolysaccharidosis type VI. Cytological, cytochemical and ultrastructural study].

作者信息

Audroin C, Lejeune F, Gaudelus J, Furioli J, Lortholary P, Joubert J, Oury-Diot M

出版信息

Nouv Rev Fr Hematol (1978). 1985;27(3):183-8.

PMID:3929230
Abstract

A cytological study of the blood and bone marrow of a child with mucopolysaccharidosis with Alder's anomaly was analysed at the optical and ultrastructural level. Morphological abnormalities of the blood and bone marrow leucocytes are defined; cytochemical and cytoenzymological abnormalities are indicated, allowing a differential diagnosis with "toxic granulations". The ultrastructural patterns of the storage cells are described compared to descriptions in the literature and the question of their mastocytic or macrophagic filiation is discussed.

摘要

对一名患有伴有阿尔德异常的黏多糖贮积症儿童的血液和骨髓进行了光学和超微结构水平的细胞学研究。确定了血液和骨髓白细胞的形态学异常;指出了细胞化学和细胞酶学异常,有助于与“毒性颗粒”进行鉴别诊断。将储存细胞的超微结构模式与文献描述进行了比较,并讨论了它们的肥大细胞或巨噬细胞谱系问题。

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