An altered membrane microfilamentous protein structure or function leads to rigidity and impaired mobility of the neutrophils, causing difficulty in their entering and exiting the circulation.
The disease is characterized by:
1. Poor polymorphonuclear leukocyte response to bacterial pyrogen injection, indicating impaired marrow release of polymorphs
3. Low or normal absolute granulocyte counts with normal phagocytic and bactericidal activity in the polymorphs
4. Normal bone marrow findings
5. Defective recruitment of polymorphs to skin windows, recruitment of monocytes normal.
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