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How I Manage Natural Killer Cell Deficiency

  • Jordan S. OrangeEmail author
How I Manage

Abstract

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.

Keywords

NK cells natural killer cell deficiency primary immunodeficiency 

Notes

Acknowledgments

I would like to thank Dr. Emily Mace who has been my close collaborator in advancing the understanding of NKD and who provided thoughtful feedback on this manuscript. My effort towards this topic was supported by NIH-NIAID R01AI120989. I would also like to thank my colleagues who have indulged me with the opportunity to think about challenging patients with them as well as those who have been collaborators in NK cell-oriented investigation. Finally, I would like to thank the patients and families who have participated in our research efforts to try and understand more about NK cells in PID and human immune defenses.

Compliance with Ethical Standards

Conflict of Interest

The author declares the following potential conflicts of interest: Royalty received for authorship of Up To Date (Wolters Kluwer Publishing) chapters on NK cell deficiency, and fees received in consultation to manufacturers of therapeutic immunoglobulin ADMA biologics (scientific advisory board membership) and Takeda.

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Authors and Affiliations

  1. 1.Department of Pediatrics, NewYork Presbyterian Morgan Stanley Children’s HospitalColumbia University Vagelos College of Physicians and SurgeonsNew YorkUSA

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