白细胞粘附缺陷
医学
免疫学
移植
吞噬细胞
干细胞
粘附
慢性肉芽肿性疾病
原发性免疫缺陷
内科学
免疫系统
生物
整合素αM
细胞生物学
化学
有机化学
CD18型
作者
Amir Ali Hamidieh,Zahra Pourpak,Kamran Alimoghaddam,Masoud Movahedi,Gholamreza Bahoush,Fatemeh Behmanesh,Mostafa Moin,Ardeshir Ghavamzadeh
标识
DOI:10.1111/j.1399-3046.2009.01239.x
摘要
LAD-I is a rare, autosomal recessive, primary immunodeficiency in which phagocyte adhesion and chemotaxis are impaired. Multiple infections in the absence of pus accumulation and persistent elevated peripheral blood neutrophil counts are the hallmark of LAD-I. Allogeneic HSCT is the only treatment proved to be potentially curative for phagocyte adhesion impairment in LAD-I. Here, we report on a case of a 30-month-old girl with LAD-I, in whom peripheral blood stem cell from a genotypically identical sibling resulted in mixed chimerism.
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