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A 38-year-old male patien t is evaluated for fatigue. He has no other specific com-plaints; he den ies fever. chills. abdomi nal pain. and urinary symptoms. Family history includes a brother who died at age of 8 years of renal failure. Complete blood count shows leukocyte count of 7 . 800/ J.LL. hemoglobin 8.9 g/dl. and platelet count 9.000/ J.LL. Creatinine is 7.9 mg/dl. LDH is 2200 U/L. The peripheral blood smear is shown below.
A diagnosis of atypical hemolytic-uremic syndrome would note what complement abnormalities?
- Normal C4, decreased C3 levels
- Decreased C4 and C3 levels
- Normal C4 and C3 levels
- Increased C4, normal C3 levels
Medicine
A. In the atypical HUS syndrome, complement tests show decreased C3 and a normal C4. This is caused by activation of the alternative complement pathway, in which C3 is cleaved but C4 is not. (NE]M. 2009;361:1676-1 687).
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