Comparative analysis of the phenotypic characteristics of neutrophils from patients with specific granule deficiency and mice deficient for C/EBPϵ protein.
| Phenotypic Characteristics . | Specific Granule Deficiency . | C/EBPϵ Knockout Mouse . |
|---|---|---|
| Clinical Presentation | Multiple bacterial infections | Bacterial infections and MDS-like disorder |
| Neutrophil morphology | Bilobed nuclei | Bilobed nuclei |
| Absent 20 granules by Wright staining | Absent 20 granules by Wright staining | |
| Absent eosinophils by Wright staining | Absent eosinophils by Wright staining | |
| Neutrophil migration | Decreased (by Rebuck skin window) | Decreased (by thioglycollate challenge) |
| Disaggregation | Decreased | Not tested |
| Phagocytosis | Normal | Decreased |
| Superoxide generation: | ||
| PMA | Decreased | Decreased |
| A23187 | Decreased | Not tested |
| Fmet-leu-phe | Normal | Not tested |
| Bactericidal activity | Decreased | Decreased |
| Phenotypic Characteristics . | Specific Granule Deficiency . | C/EBPϵ Knockout Mouse . |
|---|---|---|
| Clinical Presentation | Multiple bacterial infections | Bacterial infections and MDS-like disorder |
| Neutrophil morphology | Bilobed nuclei | Bilobed nuclei |
| Absent 20 granules by Wright staining | Absent 20 granules by Wright staining | |
| Absent eosinophils by Wright staining | Absent eosinophils by Wright staining | |
| Neutrophil migration | Decreased (by Rebuck skin window) | Decreased (by thioglycollate challenge) |
| Disaggregation | Decreased | Not tested |
| Phagocytosis | Normal | Decreased |
| Superoxide generation: | ||
| PMA | Decreased | Decreased |
| A23187 | Decreased | Not tested |
| Fmet-leu-phe | Normal | Not tested |
| Bactericidal activity | Decreased | Decreased |