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Disease Presentation, Treatment Options, and Outcomes for Myeloid Immunodeficiencies

  • Immune Deficiency and Dysregulation (. Kuo, Section Editor)
  • Published:
Current Allergy and Asthma Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

Up-to-date review on various types of immunodeficiencies with a significant myeloid component including some more recently described congenital disorders.

Recent Findings

While a number of disorders have been described in the past, genetic sequencing has led to the identification of the specific disorders and clarified their pathophysiology. Advances in genetic therapies including genetic editing should provide future treatments beyond hematopoietic stem cell transplant for patients with these rare disorders.

Summary

Neutrophils (or granulocytes) are a major contributor to infection surveillance and clearance, and defective neutrophils characteristically lead to pyogenic infections. Deficiency in numbers, either iatrogenic or congenital; functional defects; and/or inability to target to the sites of infection can all lead to serious morbidity and mortality; however, myeloid-based immunodeficiencies are not all the same. Having absent neutrophils, that is, neutropenia, has implications different to those of having dysfunctional neutrophils as will become evident as the various disorders are reviewed.

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Acknowledgements

Funding for this study was provided in part by the Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health. The content of this publication does not necessarily reflect the views or policies of the Department of Health and Human Services, nor does the mention of trade names, commercial products, or organizations imply endorsement by the U.S. Government.

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Kang, E.M. Disease Presentation, Treatment Options, and Outcomes for Myeloid Immunodeficiencies. Curr Allergy Asthma Rep 21, 14 (2021). https://doi.org/10.1007/s11882-020-00984-8

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