Modern management of phagocyte defects

Pediatr Allergy Immunol. 2017 Mar;28(2):124-134. doi: 10.1111/pai.12654. Epub 2016 Oct 13.

Abstract

Phagocytic neutrophil granulocytes are among the first immune cells active at sites of infection, forming an important first-line defense against invading microorganisms. Congenital immune defects concerning these phagocytes may be due to reduced neutrophil numbers or function. Management of affected patients depends on the type and severity of disease. Here, we provide an overview of causes and treatment of diseases associated with congenital neutropenia, as well as defects of the phagocytic respiratory burst.

Keywords: CGD; G-CSF; HSCT; Phagocyte defects; chronic granulomatous disease; congenital neutropenia; gene therapy.

Publication types

  • Review

MeSH terms

  • Animals
  • Antibiotic Prophylaxis
  • Cell Differentiation
  • Congenital Bone Marrow Failure Syndromes
  • Granulocyte Colony-Stimulating Factor / therapeutic use
  • Granulomatous Disease, Chronic / immunology*
  • Granulomatous Disease, Chronic / therapy
  • Hematopoietic Stem Cell Transplantation
  • High-Throughput Nucleotide Sequencing
  • Humans
  • Immunity, Innate
  • Immunologic Deficiency Syndromes / genetics
  • Immunologic Deficiency Syndromes / immunology*
  • Immunologic Deficiency Syndromes / therapy
  • Myelopoiesis / genetics
  • Neutropenia / congenital*
  • Neutropenia / genetics
  • Neutropenia / immunology
  • Neutropenia / therapy
  • Neutrophils / immunology*
  • Phagocytosis* / genetics
  • Primary Immunodeficiency Diseases
  • Respiratory Burst / genetics
  • Warts / genetics
  • Warts / immunology*
  • Warts / therapy

Substances

  • Granulocyte Colony-Stimulating Factor

Supplementary concepts

  • Neutropenia, Severe Congenital, Autosomal Recessive 3
  • WHIM syndrome