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Abnormal neutrophil chemotaxis in Gaucher disease
M Aker1, A Zimran, A Abrahamov
1Department of Paediatrics, Hadassah University Hospital, Ein Kerem, Jerusalem, Israel.
British Journal of Haematology
|February 1, 1993
Summary
Gaucher disease patients with intact spleens may experience recurrent infections due to impaired neutrophil function, specifically reduced migration. This neutrophil defect is linked to severe Gaucher disease and increased infection risk.
Area of Science:
- Immunology
- Hematology
- Genetics
Background:
- Gaucher disease patients often face infections, typically linked to spleen removal.
- However, some patients with intact spleens also experience recurrent pyogenic infections.
Purpose of the Study:
- To investigate neutrophil function in Gaucher disease patients with intact spleens presenting with recurrent infections.
- To determine if neutrophil dysfunction contributes to infection susceptibility in these patients.
Main Methods:
- Assessed neutrophil chemotaxis towards zymosan-activated serum and N-formyl-methionyl-leucyl-phenylalanine.
- Evaluated random neutrophil migration, adherence to nylon fibers, and oxygen radical production.
- Correlated neutrophil function with disease severity, genotype, and infection history.
Main Results:
- Nine of 29 patients showed significantly decreased neutrophil chemotaxis.
- Five of these nine patients had impaired random migration.
- Neutrophil adherence and O2- production were normal. Impaired chemotaxis correlated with severe genotypes (1448/1448, 1226/84GG) and disease severity.
- Three patients with impaired chemotaxis experienced recurrent pyogenic infections.
Conclusions:
- Neutrophil migration impairment, particularly chemotaxis and random movement, may contribute to recurrent infections in advanced Gaucher disease patients, irrespective of spleen status.
- This finding suggests a potential therapeutic target beyond splenectomy management.