自然杀伤细胞缺乏症

Hussain M, Tipu Hn, A. D, A. M, Sheikh S, Ijaz Y
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引用次数: 0

摘要

自然杀伤细胞缺乏症是一种罕见的先天免疫系统疾病。分离NK细胞缺乏症可以是典型的也可以是功能性的。在典型的自然杀伤细胞缺乏症中,NK细胞占外周血淋巴细胞的比例≤1%,而功能性NK细胞缺乏症则与发挥其功能的能力下降有关。NKD使患者更容易发生病毒感染(特别是疱疹、水痘带状疱疹、巨细胞病毒和Ebsteinbarr病毒)和恶性肿瘤。当所有其他原发性免疫缺陷的原因被排除后,反复发烧和胸部感染的患者应该怀疑这种缺陷。2岁女患者,反复胸部感染,7个月起断断续续发热,抗生素治疗效果极差,多次血培养阴性。淋巴细胞亚群分析在两个月的间隔重复和自然杀伤细胞缺乏症的最终诊断被建立。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Natural Killercell Deficiency (NKD)
Natural Killer Cell Deficiency (NKD) alone is a rare disorder of innate immune system.Isolated NK cell is deficiency can be classical or functional. In classical Natural killer cell deficiency, NK cells are ≤1% of peripheral blood lymphocytes whereas functional NK cell deficiency is associated with decrease ability to perform its function. NKD makes patients more prone to viral infections (especially with Herpes, Varicella zoster, Cytomegalo & Ebsteinbarr viruses) and malignancies. This defect should be suspected in patients with repeated fever and chest infections, when all other causes of primary immunodeficiency are ruled out. A 2-years-female patient with repeated chest infections, on off fever since the age of 7 months with very poor to antibiotics and negative blood culture on several occasions. Lymphocyte subset analysis repeated at a gap of two months and a final diagnosis of Natural killer cell deficiency was established.
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