Persistent mandibular infection in three patients with lazy and incompetent phagocyte syndromes

Insights

Lazy phagocyte syndrome, a rare immune disorder, causes recurrent infections due to defective neutrophil movement. This study identifies variants with late onset and varying severity, expanding the syndrome

Area of Science:

  • Immunology
  • Hematology

Background:

  • Recurrent orofacial infections despite antibiotic treatment suggest underlying immune dysfunction.
  • Lazy phagocyte syndrome (LPS) is characterized by impaired neutrophil mobility.

Observation:

  • Three patients presented with persistent orofacial infections and neutrophil counts at the lower normal limit.
  • Neutrophil counts did not increase with exercise or infection.
  • Abnormal chemotaxis, random mobility, and skin window tests were observed.

Findings:

  • Two patients exhibited defective intracellular killing of Candida albicans.
  • Findings align with LPS but show variations in onset and neutropenia severity.
  • A basic intrinsic defect of neutrophil movement is common across variants.

Implications:

  • LPS should be recognized to encompass diverse variants, including those with late onset and impaired phagocytic functions.
  • Understanding these variants is crucial for accurate diagnosis and management of recurrent infections.
  • Further research into the molecular basis of LPS variants is warranted.

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