Inherited Neutrophil Disorders with Infectious Manifestations

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Laboratorinė medicina. 2009,
t. 11,
Nr. 4,
p. 209 -
214

Inherited Neutrophil Disorders with Infectious Manifestations

Kristina Marcinkevičienė, Jurgita Songailienė, Rėda Matuzevičienė

 

Summary

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.

 

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