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Beneficial effect of granulocyte transfusions in patients with defects in granulocyte function and severe infections
Insights
Granulocyte transfusions effectively managed severe infections in a child with a congenital defect and an adult with an acquired granulocyte function defect, resolving pneumonia and recurrent infections.
Area of Science:
- Immunology
- Hematology
Background:
- Severe infections can arise from congenital or acquired defects in granulocyte function.
- Impaired neutrophil function affects the body's ability to combat bacterial and fungal pathogens.
- Recurrent infections pose a significant health risk, especially in pediatric and immunocompromised populations.
Abstract:
A 3-year-old boy (patient A) with a congenital and a 24-year-old man (patient B) with an acquired granulocyte function defect received supportive granulocyte transfusions for the management of severe infections. The boy had suffered from recurrent infections since bith. His granulocytes showed in vitro almost no chemotactic responsiveness, an impaired phagocytosis and reduced intracellular killing of Candida albicans. Family studies suggested that it was an inherited autosomal recessive defect. The child developed a Pseudomonas pneumonia at the age of 3 years, which did not respond to antibiotic therapy. Granulocyte transfusions were then started and soon after the fever and pneumonia disappeared. Patient B showed the haematological signs of a preleukaemic state. He had 3 recurrent episodes of furunculosis which led each time to cellulitis and septic temperatures accompanied by symptoms of an enterocolitis. Tests of granulocyte function in vitro showed reduced intracellular killing of Staphylococcus aureus. Granulocyte transfusions were started, since no clinical improvement could be attained by antibiotics. With transfusion therapy, fever, cellulitis and enterocolitis disappeared each time.