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Pathology · Inflammation: complement deficiencies
Sample itemA 19-year-old man has his second episode of meningococcal meningitis within two years. He has no splenic abnormality and normal immunoglobulin levels, but total haemolytic complement activity (CH50) is nearly absent. Which deficiency best explains this?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
C. Terminal complement components (C5 to C9) forming the membrane attack complex
Deficiency of the terminal components C5 to C9 prevents formation of the membrane attack complex, which is needed to lyse Neisseria. The patients have recurrent meningococcal or disseminated gonococcal infection and a low CH50. Deficiency of C3 causes severe recurrent infections by many encapsulated pyogenic bacteria from early childhood.
Option by option
A. Decay-accelerating factor (CD55)
Decay-accelerating factor deficiency causes complement-mediated lysis of red cells in paroxysmal nocturnal haemoglobinuria.
B. Mannose-binding lectin, with an intact classical pathway
Mannose-binding lectin deficiency leaves the classical pathway and CH50 normal, and gives at most a mild infection tendency in infancy.
C. Terminal complement components (C5 to C9) forming the membrane attack complex (correct)
Correct. Without the membrane attack complex Neisseria species escape complement-mediated killing, giving recurrent infection.
D. C1 esterase inhibitor
C1 inhibitor deficiency causes hereditary angioedema, not recurrent meningitis.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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