Polymorphonuclear Dysfunction in Hematology

Polymorphonuclear cells defend against bacterial and fungal pathogens through chemotaxis, phagocytosis, and oxidative burst. Functional defects include leukocyte adhesion deficiency from CD18 mutation with delayed umbilical cord separation and lack of pus formation, chronic granulomatous disease from NADPH oxidase defect with severe catalase positive infections, Chediak Higashi syndrome with giant granules and partial albinism, specific granule deficiency, and hyper IgE syndrome.

Diagnosis combines clinical pattern of recurrent skin abscesses, pneumonias, and lymphadenitis with laboratory tests of dihydrorhodamine flow cytometry for oxidative burst, CD11b CD18 expression, neutrophil chemotaxis assays, microbicidal assays, and genetic testing for confirmed mutations. Acquired dysfunction may be caused by diabetes, alcohol, glucocorticoids, hemodialysis, and severe burns.

Management uses targeted antimicrobial prophylaxis with trimethoprim sulfamethoxazole in chronic granulomatous disease, itraconazole for fungal prevention, interferon gamma adjunct in selected forms, surgical drainage of abscesses, and aggressive treatment of infections. Allogeneic hematopoietic stem cell transplantation provides definitive cure in chronic granulomatous disease, leukocyte adhesion deficiency, and Chediak Higashi syndrome with HLA matched donor.