Complement Disorders PDF
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Ross University School of Medicine
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Summary
This document discusses complement disorders, including early and terminal complement deficiencies, and complement regulatory protein deficiencies. It explains the causes, characteristics, and potential consequences of these disorders, such as recurrent infections and thrombosis.
Full Transcript
IMMUNOLOGY ` IMMUNOLOGY—IMMUNE RESPONSES SEC TION II 105 Complement disorders Complement protein deficiencies Early complement deficiencies (C1–C4) risk of severe, recurrent pyogenic sinus and respiratory tract infections. C3b used in clearance of antigen-antibody complexes risk of SLE (think...
IMMUNOLOGY ` IMMUNOLOGY—IMMUNE RESPONSES SEC TION II 105 Complement disorders Complement protein deficiencies Early complement deficiencies (C1–C4) risk of severe, recurrent pyogenic sinus and respiratory tract infections. C3b used in clearance of antigen-antibody complexes risk of SLE (think SLEarly). Terminal complement deficiencies (C5–C9) susceptibility to recurrent Neisseria bacteremia. Complement regulatory protein deficiencies C1 esterase inhibitor deficiency Causes hereditary angioedema due to unregulated activation of kallikrein bradykinin. Characterized by C4 levels. ACE inhibitors are contraindicated (also bradykinin). Paroxysmal nocturnal hemoglobinuria A defect in the PIGA gene prevents the formation of glycosylphosphatidylinositol (GPI) anchors for complement inhibitors, such as decay-accelerating factor (DAF/CD55) and membrane inhibitor of reactive lysis (MIRL/CD59). Causes complement-mediated intravascular hemolysis haptoglobin, dark urine A. Can cause atypical venous thrombosis (eg, Budd-Chiari syndrome; portal vein, cerebral, or dermal thrombosis). A
