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Published on: November 27, 2014
Persistent mandibular infection in three patients with lazy and incompetent phagocyte syndromes
Abstract:
Three patients with the lazy phagocyte syndrome suffered from recurrent infections in the orofacial region which persisted despite treatment with antibiotics. All had neutrophil counts at the lower normal limits which did not increase after strenuous exercise or rise during infections. Tests of chemotaxis, random mobility and the skin window were abnormal. Ingestion and intracellular killing, however, were normal in one patient and abnormal in two. The findings are compatible with the lazy leucocyte syndrome but the symptoms were not manifest in early childhood as originally described by Miller et al. (1971), and the neutrophil counts were not as low as in his patients. Two of our patients also differed in showing a defective intracellular killing for Candida albicans. The lazy leucocyte syndrome should now be recognized to include different variants, some with late onset, a range in the degree of neutropenia, and some with defective ingestion and killing, though all possess a basic intrinsic defect of movement.
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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