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 Primary Immune Deficiencies: Neutrophil ProblemsAs mentioned in Our Immune System: The Basics, neutrophils are granulocytes (white blood cells) that are important for protection against infections. These front line fighters against infection release chemicals such as hydrogen peroxide to kill germs or clean up wounds. Increased risk of infection can happen when the body does not have enough neutrophils in the blood stream(neutropenia); when neutrophils cannot travel through the bloodstream to sites of infection efficiently; or when the neutrophils are unable to make oxidants such as hydrogen peroxide to kill infecting microbes (Chronic Granulomatous Disease). There are many reasons for neutropenia to occur, including autoimmune conditions, medications and viral infections. Some PIDD can be associated with neutropenia as a feature. Neutropenia can be mild or severe, and acute or chronic, depending on the underlying cause. Infections happen frequently and can be quite serious in patients with neutropenia. Skin infections, mouth ulcers, and gum disease are sometimes seen. Often infections require IV antibiotics to clear. In CGD, the numbers of neutrophils are generally normal, but the function is impaired. Neutrophils in CGD are unable to make hydrogen peroxide which is important in killing certain bacteria and fungi such as Staphylococcus, Serratia, Nocardia, Burkholderia and Aspergillus. These germs cause severe infections in patients with CGD that can include skin or bone infections and abscesses in the internal organs (lungs, liver, brain). Poor wound healing and an inflammatory condition known as granuloma can also be complications of CGD. Aside from the defective neutrophil function in CGD, the rest of the immune system is normal. Thus people with CGD can be generally healthy until they become infected with one of these germs which unfortunately cause prolonged hospitalizations for treatment. Special preventative antibiotics are a mainstay of treatment and greatly reduce the chances of infection with these organisms. Antibiotic treatment of severe infections in CGD generally requires a much longer amount of time than in healthy individuals. The most common form of CGD is genetically inherited in an "X-linked" manner, but there are also "autosomal recessive" forms of CGD. Bone marrow transplant is a treatment option for some people with CGD. More information about CGD (Immune Deficiency Foundation publication). |
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