Immunodeficiencies associated with deficiency of phagocytic function

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A. Primary

The majority of abnormalities in this group of diseases is due to the weakening ability to recognize and / or kill extra- and intracellular pathogens.

1. Chronic granulomatous disease. The predominant type of inheritance is X-linked (80% of patients are males), but there is also an autosomal recessive form of the disease. The basis of pathogenesis is the weakening of the bactericidal properties of neutrophils because of their inability to produce active oxygen species necessary for oxygen-dependent killing of phagocytosed microorganisms. Clinically, the disease manifests itselymphocyte in the form of recurrent infections caused by microorganisms that produce catalase (Staph. Aureus, Serratia, Escherichia, Pseudomonas). Of great etiological significance are different types of Aspergillus, causing pneumonia or disseminated infections, and Candida, which affects predominantly soft tissues. The disease usually begins in early childhood, but occasionally its manifestation is delayed until adolescence. The clinical picture includes delayed physical development, purulent lymphadenitis, hepatosplenomegaly, purulent skin and subcutaneous tissue infections, pneumonia, liver abscesses and hemogram changes indicating chronic infections. There are also rhinitis, dermatitis, diarrhea, perianal abscesses, stomatitis, osteomyelitis, brain abscesses, impaired gastrointestinal tract and genitourinary tract (formation of granulomas). Laboratory diagnostics is based on chemiluminescence and NST tests. The courses of antibiotic therapy and interferon g-therapy

2. Syndrome of "lazy" leukocytes and Schwartzman's syndrome. The basis of the pathogenesis of these conditions is the defects in the genes of membrane adhesion molecules, which leads to marked disturbances in neutrophil chemotaxis and monocytes / macrophages, as well as their interactions with other types of cells. An example is phenotypically similar lesions that develop as a result of hereditary defects in the expression of β2 integrins (their common chain, CD18) and carbohydrate determinants of L (CD15) recognized by selectin L (CD62L).

B. Secondary

Defects of phagocytic function can also be the result of various factors (chemotherapy, immunosuppressants, glucocorticoids, tumors, etc.), the effect of which was considered in the previous sections.