Inherited Neutrophil Disorders with Infectious Manifestations
Kristina Marcinkevičienė, Jurgita Songailienė, Rėda Matuzevičienė
The immune system is the system of the organs, tissues, cells and molecules which is protecting organism from harmful microorganisms, foreign proteins, helps cure of old, damaged and ill cells of the organism. There are two kinds of immunity - innate (not spe - cific) and acquired (specific). Phagocitic cells (neutrophils, monocytes and macrophages), cells releasing inflammatory mediators (eosinophils, basophils and mastocytes), natural killer cells (NK) participate in the innate immune reactions. Antigen specific T and B lymphocytes proliferation proceed in the acquired immune response. Inherited defects in macrophage function and neutrophil function are not common. Many of the inherited neutrophil dis orders are as so ci ated with neutropenia. The result of this are frequent infections. This review describes some groups of inherited diseases, which manifest in infections (congenital neutropenias, defects of intracellular killing, defects of adhesion, defects in the formalion and function of neutri- phil granules and congenital metabolic diseases). From congenital metabolic diseases we will review cystic fibrosis, congenital glycogen storage disease Ib, congenital disorder of glycosilation Ia, congenital adenosine deaminase deficiency, congenital galactosemia, congenital glutaric aciduria type 1.
During these congenital metabolic diseases various infections manifest through neutropenia, increased apoptosis of neutrophils, bactericidial function of neutrophils defect, leucocyte adhesion deficiency, neutrophil initial and secondary granule deficiency, various enzyme deficiencies.
In the review there are refered several congenital disorders and groups of diseases, which manifest with recurrent infections. There are a lot of different mechanisms of causation of the diseases, but manifestation with infections is associating them to one group.
Keywords: neutrophils, phagocytosis defect, infection, neutropenia.