Natural killer (NK) cell deficiency (NKD) is a subset of primary immunodeficiency disorders (PID) in which an abnormality of NK cells represents a major immunological defect resulting in the patient's clinical immunodeficiency. This is distinct from a much larger group of PIDs that include an NK cell abnormality as a minor component of the immunodeficiency. Patients with NKD most frequently have atypical consequences of herpesviral infections. There are now 6 genes that have been ascribed to causing NKD, some exclusively and others that also cause other known immunodeficiencies. This list has grown in recent years and as such the mechanistic and molecular clarity around what defines an NKD is an emerging and important field of research. Continued increased clarity will allow for more rational approaches to the patients themselves from a therapeutic standpoint. Having evaluated numerous individuals for NKD, I share my perspective on approaching the diagnosis and managing these patients.

中文翻译：

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我如何处理自然杀伤细胞缺乏症。

自然杀伤（NK）细胞缺乏症（NKD）是原发性免疫缺陷疾病（PID）的子集，其中NK细胞异常代表导致患者临床免疫缺陷的主要免疫缺陷。这不同于更大的一组PID，这些PID包括NK细胞异常作为免疫缺陷的次要成分。NKD患者最常出现疱疹病毒感染的非典型后果。现在有6个基因被归因于引起NKD，其中一些基因排他性地导致另一些已知的免疫缺陷。近年来，这个列表已经增长，因此围绕NKD定义的机理和分子清晰度是一个新兴且重要的研究领域。从治疗的观点来看，持续增加的清晰度将允许对患者自身采取更合理的方法。在评估了许多人的NKD后，我分享了对这些患者进行诊断和治疗的观点。