title: [Primary and secondary immunodeficiencies].
abstract: Primary Immunodeficiencies (PID) are rare, genetically determined diseases that cause dysfunctional immunity, clinically presenting as susceptibility to infection but also as autoimmunity due to deficient immune tolerance. More than 180 PID have been described to date. To diagnose a PID, secondary causes of immunodeficiency have to be excluded which can be especially challenging in the adult population. PID associated with hypogammaglobulinemia are treated by immunoglobulin replacement therapy, while the correction of the underlying cellular immune defects is often not possible. This review focuses on PID and secondary causes of immunodeficiency that occur in the adult population and discusses patho-mechanisms of select immunodeficiencies and associated immune dysregulation.
authors.affiliation: Hämatologie, Medizinische Universitätsklinik, Kantonsspital Aarau AG.
authors.forename: Nathan
authors.initials: N
authors.lastname: Cantoni
authors.affiliation: Sprechstunde für Primäre Immundefizienz, Medizinische Poliklinik und Immunodeficiency Laboratory, Departement Biomedizin, Universitätsspital Basel.
authors.forename: Mike
authors.initials: M
authors.lastname: Recher
mesh_terms: D015551:Autoimmunity / Q000276:immunology*; D006801:Humans; D007108:Immune Tolerance / Q000276:immunology*; D016867:Immunocompromised Host / Q000276:immunology*; D007153:Immunologic Deficiency Syndromes / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D012307:Risk Factors
chemical_list: D007166:Immunosuppressive Agents