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A 27yo male has noticed that his urine was cola-colored for the past several day

ID: 3478806 • Letter: A

Question

A 27yo male has noticed that his urine was cola-colored for the past
several days with accompanying severe abdominal pain. Physical exam, blood
tests and urinalysis show that he is anemic as a result of intravascular hemolysis
(lysis of the RBC). Other tests rule out autoimmune hemolytic anemia. Flow
cytometric analysis of blood cells reveals the absence of CD59 on half of his RBC.
CD59 is a complement control protein that normally prevents the recruitment of
C9 on human cells.
Which of the following treatment options would you recommend and why?
a. Inhibit production of anaphylatoxins to decrease the inflammation that may be leading to
hemolytic anemia
b. Inhibit the production of C3 convertase
c. Inhibit C5 with an anti-C5 antibody

Explanation / Answer

CD59 is a compliment control protein. It prevents the recruitment of C9 on human cells. The patient is showing signs and symptoms of intravascular hemolysis and his red blood cells are deficient in CD59. C5 is the immediate common precursor needed to form membrane attack complex which is responsible for intravascular hemolysis. Therefore, the appropriate treatment here would be to inhibit C5 with an anti-C5 antibody (option c).

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