Table 2

Relation of presenting features and clinical outcomes to ADAMTS13 levels

Presenting features and clinical outcomesADAMTS13 activity
P
10% or more, n = 201< 10%, n = 60
Demographic features    
    Age, y 51 (0.6, 86) 41 (9, 72) .002 
    Race, % black 16 35 .001 
    Sex, % female 63 82 .007 
    Obesity, BMI, kg/m2 24.7 (13.6, 41.1) 30.3 (15.3, 51.4) < .001 
Presenting clinical and laboratory features    
    Severe neurologic abnormalities, % 44 50 .435 
    Hematocrit, % 22 (9, 40) 21 (13, 33) .130 
    Platelets, /μL 22 (1, 129) 11 (2, 101) < .001 
    LDH, U/L 1090 (114, 12 587) 1407 (256, 3909) .059 
    Creatinine, mg/dL 4.6 (0.2, 33) 1.6 (0.7, 6.6) < .001 
    Acute renal failure, % 54 10 < .001 
Clinical outcomes    
    Death, % 32 22 .113 
    Plasma exchange, no. 8 (0, 71) 19 (2, 79) < .001 
    Relapse, %* 4 (5/136) 34 (16/47) < .001 
Presenting features and clinical outcomesADAMTS13 activity
P
10% or more, n = 201< 10%, n = 60
Demographic features    
    Age, y 51 (0.6, 86) 41 (9, 72) .002 
    Race, % black 16 35 .001 
    Sex, % female 63 82 .007 
    Obesity, BMI, kg/m2 24.7 (13.6, 41.1) 30.3 (15.3, 51.4) < .001 
Presenting clinical and laboratory features    
    Severe neurologic abnormalities, % 44 50 .435 
    Hematocrit, % 22 (9, 40) 21 (13, 33) .130 
    Platelets, /μL 22 (1, 129) 11 (2, 101) < .001 
    LDH, U/L 1090 (114, 12 587) 1407 (256, 3909) .059 
    Creatinine, mg/dL 4.6 (0.2, 33) 1.6 (0.7, 6.6) < .001 
    Acute renal failure, % 54 10 < .001 
Clinical outcomes    
    Death, % 32 22 .113 
    Plasma exchange, no. 8 (0, 71) 19 (2, 79) < .001 
    Relapse, %* 4 (5/136) 34 (16/47) < .001 

Relation of demographic, clinical, and laboratory features and clinical outcomes to ADAMTS13 levels. Continuous data are presented as median values with the minimum and maximum values in parentheses. Severe neurologic abnormalities are defined as coma, stroke, seizure, or focal neurologic signs. Laboratory data are the most abnormal values at the time of diagnosis, defined as the day of the first plasma exchange treatment, ± 7 days to account for transient changes resulting from transfusion or worsening renal failure. Acute renal failure is defined as either (1) an increasing serum creatinine (≥ 44.5 μmol/L [≥ 0.5 mg/dL] per day for 2 consecutive days) or (2) serum creatinine ≥ 353.6 μmol/L (≥ 4.0 mg/dL) plus dialysis that began within 7 days of diagnosis. The number of plasma exchange treatments and the percent of patients with relapse were analyzed only in surviving patients.

BMI indicates body mass index; and LDH, lactate dehydrogenase.

*

Number in parentheses represents the number of patients who relapsed divided by the number of patients who survived.

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