Table 7

Group clinical course data for patients with ADAMTS13 activity < 10% at their initial diagnosis

Relapse after remission sampleNo relapse after remission sample
ADAMTS13 activity during remission (n = 7) (n = 33) 
    1 or more samples < 10% 1 (14%) 5 (15%) 
    1 or more samples 10% to 20% 3 (43%) 3 (9%) 
    1 or more samples 21% to 49% 1 (14%) 5 (15%) 
    All samples 50% or more 2 (29%) 20 (61%) 
ADAMTS13 inhibitor during remission Inhibitor measured (n = 4) Inhibitor measured (n = 8) 
    1 or more samples with a strong inhibitor (2 or more BU/mL) 1 (25%) 4 (50%) 
    1 or more samples with a mild-moderate inhibitor (more than 0 to less than 2) 2 (50%) 2 (25%) 
    All samples with no inhibitor 1 (25%) 2 (25%) 
Relapse after remission sampleNo relapse after remission sample
ADAMTS13 activity during remission (n = 7) (n = 33) 
    1 or more samples < 10% 1 (14%) 5 (15%) 
    1 or more samples 10% to 20% 3 (43%) 3 (9%) 
    1 or more samples 21% to 49% 1 (14%) 5 (15%) 
    All samples 50% or more 2 (29%) 20 (61%) 
ADAMTS13 inhibitor during remission Inhibitor measured (n = 4) Inhibitor measured (n = 8) 
    1 or more samples with a strong inhibitor (2 or more BU/mL) 1 (25%) 4 (50%) 
    1 or more samples with a mild-moderate inhibitor (more than 0 to less than 2) 2 (50%) 2 (25%) 
    All samples with no inhibitor 1 (25%) 2 (25%) 

Data are presented for the 37 patients who had ADAMTS13 activity < 10% at the time of their initial diagnosis of TTP and who also had ADAMTS13 activity measured during clinical remission, documented by clinical evaluation, platelet count, and hematocrit. ADAMTS13 activity and inhibitor were measured by FRETS-VWF73 method. Patient 18 was omitted from this analysis because of the uncertain cause of her death.

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