NK cell deficiency syndromes: Treatment
- Jordan S Orange, MD, PhD
Jordan S Orange, MD, PhD
- Section Editor — Immunology and Immunodeficiency
- Professor of Pediatrics
- Chief of Immunology, Allergy, and Rheumatology
- Baylor College of Medicine
- Texas Children's Hospital
Natural killer (NK) cell deficiency syndromes are rare disorders in which NK cells are absent, deficient, or dysfunctional, in the absence of any other identifiable immunodeficiency, genetic disorder, or medication known to affect NK cells. These disorders are categorized as classical NK cell deficiency (CNKD) and functional NK cell deficiency (FNKD).
●In CNKD, NK cell developmental status or survival is abnormal, often leading to decreased NK cell numbers in the peripheral blood.
●In FNKD, NK cells are appropriately developed and present but are functionally impaired.
The management of patients with these disorders is discussed here. The biology of NK cells, the clinical manifestations of NK cell disorders, and the evaluation of patients suspected of having NK cell defects are presented separately. (See "NK cell deficiency syndromes: Clinical manifestations and diagnosis".)
Therapy for patients with NK cell deficiency syndromes is largely empiric, due to the small numbers of cases described in the literature. Active infections must be identified and treated aggressively, as with any immunodeficiency. (See "Primary immunodeficiency: Overview of management".)To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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