Department of Pediatrics, Section of Allergy and Immunology, LSU Health Sciences Center, Shreveport, Louisiana 71130, USA.
Ann Allergy Asthma Immunol. 2010 Apr;104(4):286-92. doi: 10.1016/j.anai.2010.01.021.
To compare autoimmune lymphoproliferative syndrome (ALPS) and Stevens-Johnson syndrome (SJS) with respect to the defects in Fas- and Fas ligand (FasL)-mediated apoptosis.
Selected reviews, case reports, and original studies were searched in PubMed and MEDLINE for the keywords ALPS, SJS, Fas, FasL, and apoptosis.
Case reports of ALPS and SJS were selected as examples of Fas- and FasL-mediated diseases. In addition, we selected articles that examined the pathophysiology of apoptosis in the context of Fas-FasL interaction.
Failure to initiate apoptosis of abnormal T lymphocytes occurs in such diseases as ALPS, leading to the accumulation of double negative T cells with an increase in autoimmunity. In contrast to apoptotic failure, SJS is associated with a pathological increase in programmed keratinocyte cell death.
The consequences of dysregulated Fas- and FasL-mediated apoptosis leads to self-reactivity, malignant transformation, and immune dysfunction. An understanding of underlying mechanisms and qualitative assessment of Fas and FasL may have clinical benefits when control of these homeostatic mechanisms is in question.
比较自身免疫性淋巴组织增生综合征（ALPS）和史蒂文斯-约翰逊综合征（SJS）在 Fas 和 Fas 配体（FasL）介导的细胞凋亡缺陷方面的差异。
在 PubMed 和 MEDLINE 中使用“ALPS”、“SJS”、“Fas”、“FasL”和“apoptosis”等关键词搜索了选定的综述、病例报告和原始研究。
选择 ALPS 和 SJS 的病例报告作为 Fas 和 FasL 介导疾病的例子。此外，我们选择了研究 Fas-FasL 相互作用背景下细胞凋亡病理生理学的文章。
在 ALPS 等疾病中，异常 T 淋巴细胞不能起始凋亡，导致自身免疫增加的双阴性 T 细胞积累。与凋亡失败相反，SJS 与程序性角质形成细胞死亡的病理性增加有关。
Fas 和 FasL 介导的细胞凋亡失调导致自身反应性、恶性转化和免疫功能障碍。当这些体内平衡机制受到质疑时，对 Fas 和 FasL 的潜在机制和定性评估的理解可能具有临床益处。
