Complement Deficiencies

• Primarily manifested by autoimmune phenomena such as lupus

  Deficiency of classic pathway
• Leads to recurrent infections with gram-positive encapsulated organisms:

  Deficiency of C2 and C3
• Most common deficiency, atherosclerosis

  C2
• Most severe deficiency associated with life threatening infections:

  C3
• Symptoms include recurrent serious systemic infections, especially Neisseria meningitidis and Neisseria gonorrhea

  Deficiency of Membrane Attack Complex
• C5, 6, 7, 8

  Deficiency of Membrane Attack Complex
• C9 deficiency

  no known disease
• Hereditary angioedema (HAE)

  Deficiency of C 1 esterase inhibitor (Cl INH)
• Urinary histamine levels and serum Cl levels are elevated during attacks, whereas serum CH5O, C4 and C2 are decreased. Between attacks, C4 is always low, whereas C2 levels are normal
• In Deficiency of C 1 esterase inhibitor (Cl INH), First line therapy is recombinant C 1 inhibitor concentrate
• Paroxysmal Nocturnal Hemoglobinuria

  CD55 (DAF) & CD59 (MIRL) deficiency
• answer #1: classical

  
• answer #2: lectin

  
• answer #3: alternative

  
• answer #4: low

  
• answer #5: normal

  
• answer #6: normal

  
• answer #7: low

  
• answer #8: low

  
• answer #9: normal

  
• answer #10: normal

  
• answer #11: low

  
• answer #12: normal

  
• answer #13: normal

  
• answer #14: normal

  
• answer #15: low

  
• answer #16: low

  
• answer #17: low

  
• answer #18: low

  
• answer #19: low

  
• answer #20: low

  
• answer #21: low

  
• answer #22: low

  
• answer #23: low

  
• answer #24: low

  
• answer #25: low

  
• answer #26: low

  
• answer #27: low

  
• Pneumococcal diseases:

  MBL

  MASP
• Recurrent pyogenic infections:

  Factor H

  Factor I