Table 1.

Comparison of presenting clinical and laboratory findings in iTTP and aHUS

Immune TTP6,7 aHUS (adult)8,9,44
Adult or childhood onset More common in adults
(Congenital TTP more common in children) 
Similar prevalence of adults and children 
MAHA with thrombocytopenia 100% ~85% 
Neurologic findings Overall, 67%-78%
Seizure, 7%-8%
Coma, 3%-10%
Stroke, 5%-10% 
Overall, 8%-43% 
Thrombocytopenia More severe
Median: 10-15 × 109/L 
Less severe
Median: 57-118 × 109/L 
Renal dysfunction Less severe
Median serum creatinine: 1.09-1.3  mg/dL 
More severe
Median serum creatinine: 2.9-4.6  mg/dL 
Severe (or malignant) hypertension N/A, uncommon Common, 8%-54% 
Cardiac involvement 42% 19% 
Fever 10%-24% Not reported 
Abdominal symptoms 35% 15% 
ADAMTS13 activity (%), median (range)16  5 (0-11) 66.5 (12-119) 
Immune TTP6,7 aHUS (adult)8,9,44
Adult or childhood onset More common in adults
(Congenital TTP more common in children) 
Similar prevalence of adults and children 
MAHA with thrombocytopenia 100% ~85% 
Neurologic findings Overall, 67%-78%
Seizure, 7%-8%
Coma, 3%-10%
Stroke, 5%-10% 
Overall, 8%-43% 
Thrombocytopenia More severe
Median: 10-15 × 109/L 
Less severe
Median: 57-118 × 109/L 
Renal dysfunction Less severe
Median serum creatinine: 1.09-1.3  mg/dL 
More severe
Median serum creatinine: 2.9-4.6  mg/dL 
Severe (or malignant) hypertension N/A, uncommon Common, 8%-54% 
Cardiac involvement 42% 19% 
Fever 10%-24% Not reported 
Abdominal symptoms 35% 15% 
ADAMTS13 activity (%), median (range)16  5 (0-11) 66.5 (12-119) 
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