Abstract:

Primary immunodefi ciencies (PIDs) are inherited disorders in which one or several components of immune system are aff ected. PIDs comprises more than 200 diff erent disorders and the number of newly recognized defi ciencies is still increasing. PIDs are characterized by an increased susceptibility to infections, allergy, autoimmune disorders, autoinfl ammation, lymphoproliferation, hemophagocytosis, and malignancies. Infectious diseases are a major source of morbiditiy and mortality in the majority of patients with PIDs. Th erefore, prevention and treatment of infectious disease are important as curative treatment. Basis for the treatment of PIDs include; reduce exposure to infectious agents, antimicrobial prophylaxis, vaccine applications, aggressive treatment of infections, regulation of the immune system with various agents (eg. immunoglobulin), and reconstitution of the immune system with hematopoietic stem cell transplantation or gene therapy. In this review, treatment modalities of PIDs were aimed to be reviewed.

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