Table 1.

Demographic data and clinical presentation of each TTP patient

No.
Age, y
Race and sex
Etiology
Episodes
Plt, × 109/L
Hct
LDH, U/L
Dialysis
Cr, μM
Fever
CNS symptoms and signs
% ADAMTS13
Inhibitor, U/mL
Other treatments
Outcome
F/U, mo
1   38   AAF   Idiopathic   Initial   10   0.314   1836   No   123.76   No   None   < 5   Neg   None   Res-Rem   13  
2   62   AAF   Idiopathic   Initial   8   0.165   2090   No   88.4   Yes   Fatigue and dizziness   < 5   Neg   P, V, S   Res-Rem   13  
3   39   WF   Idiopathic   Initial   20   0.156   1818   No   88.4   No   None   < 5   Neg   P   Res-Rem   33  
4   47   AAF   Idiopathic   Initial   23   0.18   769   No   79.56   No   None   < 5   Neg   None   Res-Rem   21  
5   77   AAF   Idiopathic   Multi   11   0.266   408   No   132.6   Yes   Briefly aphasic   16   Neg   IVIg, P   Res-Rem   32  
6   79   AAF   Idiopathic   Initial   7   0.274   1660   No   123.76   No   AMS   < 5   Neg   P, CVP   Res-Rem   25  
7   56   AAF   Idiopathic   Initial   19   0.29   654   No   97.24   No   AMS   < 5   Neg   P, V   Res-Rem   22  
8   50   AAF   Idiopathic   Initial   6   0.326   627   No   88.4   No   Mild headache   < 5   Neg   None   Res-Rem   17  
9   62   AAF   Idiopathic   Initial   8   0.213   1062   No   150.28   Yes   AMS   < 5   Neg   P, V, S   Res-Rem   8  
10   62   WF   Idiopathic   Multi   12   0.304   1084   No   97.24   Yes   None   < 5   6.5*  CVP, S   Rem-Rel-Rem   35  
11   52   AAF   Idiopathic   Multi   20   0.20   866   No   97.24   No   None   < 5   8*  P, S, CVP   Rem-Rel-Died   2† 
12   44   AAF   Idiopathic   Multi   15   0.23   695   No   97.24   Yes   Stroke, blindness   < 5   10*  CVP, S, CSA, rituximab   Rem-Rel-Rem   48  
13   21   WF   Idiopathic   Initial   9   0.129   1489   No   132.6   No   AMS   < 5   5*  P   Res-Rem   18  
14   26   WF   Idiopathic   Multi   18   0.25   680   No   79.56   Yes   Headache   < 5   1*  P   Rem-Rel-Rem   16  
15   42   WF   Idiopathic   Initial   42   0.249   1584   No   159.12   No   None   25   Neg   P   Res-Rem   32  
16   42   AAF   Idiopathic   Initial   16   0.238   1262   No   150.28   No   None   14   Neg   None   Res-Rem   32  
17   73   AAF   Idiopathic   Multi   35   0.28   939   No   61.88   No   None   < 5   5*  P, S   Rem-Rel-Died   0.03  
18   46   AAF   Idiopathic   Multi   40   0.32   409   No   238.68   No   Aphasia   < 5   Neg   P, V, S   Rem-Rel-Died   0.5  
19   37   WF   Idiopathic   Multi   80   0.37   428   Yes   380.12   No   AMS   46   Neg   None   Rem-Rel-Rem   22  
20   59   WF   Idiopathic   Initial   47   0.247   638   No   265.2   No   None   < 5   1*  None   Res-Rem   7  
21   54   WF   HSCT   Initial   25   0.326   1150   No   44.2   No   AMS   41   Neg   None   NR-Died   0.6  
22   54   WF   HSCT   Initial   11   0.225   463   No   132.6   No   AMS   16   Neg   None   Res-Rem   25  
23   19   WM   HSCT   Initial   34   0.283   328   No   70.72   Yes   None   56   Neg   P   Res-Rem   30  
24   45   AsiF   HSCT   Initial   15   0.296   1483   No   141.44   No   None   66   Neg   None   NR   25  
25   44   WM   HSCT   Initial   8   0.31   1902   Yes   565.76   Yes   Slurred speech   64   Neg   P   NR-Died   1.3  
26   25   WM   HSCT   Initial   13   0.236   1678   No   424.32   No   AMS   84   Neg   None   NR-Died   ?  
27   30   WM   HSCT   Multi   16   0.263   1362   No   291.72   Yes   AMS   32   Neg   P   Rem-Rel-Died   3  
28   47   WF   HSCT   Initial   4   0.261   648   Yes   194.48   No   AMS   32   Neg   P   Res-Rem   35  
29   69   WF   FK506   Initial   23   0.252   832   No   150.28   Yes   None   34   Neg   P   Res-Died   1.3  
30   44   WM   Cancer   Initial   147   0.25   839   No   344.76   No   None   66   Neg   P   NR-Died   2  
31   52   AAF   Cancer   Multi   18   0.24   1477   Yes   238.68   Yes   Syncope   48   Neg   P   Res-Died   5  
32   59   WF   Mitomycin   Initial   2   0.19   2383   Yes   97.24   No   AMS   29   Neg   None   NR-Died   36  
33   74   WM   Clopidogrel   Multi   26   0.25   263   Yes   247.52   Yes   None   96   Neg   P, V   NR-Died   1.6  
34   16   AAM   Lupus   Initial   41   0.238   ND   No   176.8   No   None   52   Neg   P, S, V   NR-Died   2  
35   17   AAF   SCD   Initial   39   0.221   28000   Yes   309.4   No   AMS   74   Neg   None   Res-Rem   19  
36   29   WF   Postpartum   Initial   36   0.28   2401   No   256.36   No   None   48   Neg   None   Res-Rem   32  
37
 		 36
 		 WF
 		 Pregnancy
 		 Initial
 		 46
 		 0.266
 		 1118
 		 No
 		 53.04
 		 Yes
 		 None
 		 116
 		 Neg
 		 None
 		 Res-Rem
 		 28
 
No.
Age, y
Race and sex
Etiology
Episodes
Plt, × 109/L
Hct
LDH, U/L
Dialysis
Cr, μM
Fever
CNS symptoms and signs
% ADAMTS13
Inhibitor, U/mL
Other treatments
Outcome
F/U, mo
1   38   AAF   Idiopathic   Initial   10   0.314   1836   No   123.76   No   None   < 5   Neg   None   Res-Rem   13  
2   62   AAF   Idiopathic   Initial   8   0.165   2090   No   88.4   Yes   Fatigue and dizziness   < 5   Neg   P, V, S   Res-Rem   13  
3   39   WF   Idiopathic   Initial   20   0.156   1818   No   88.4   No   None   < 5   Neg   P   Res-Rem   33  
4   47   AAF   Idiopathic   Initial   23   0.18   769   No   79.56   No   None   < 5   Neg   None   Res-Rem   21  
5   77   AAF   Idiopathic   Multi   11   0.266   408   No   132.6   Yes   Briefly aphasic   16   Neg   IVIg, P   Res-Rem   32  
6   79   AAF   Idiopathic   Initial   7   0.274   1660   No   123.76   No   AMS   < 5   Neg   P, CVP   Res-Rem   25  
7   56   AAF   Idiopathic   Initial   19   0.29   654   No   97.24   No   AMS   < 5   Neg   P, V   Res-Rem   22  
8   50   AAF   Idiopathic   Initial   6   0.326   627   No   88.4   No   Mild headache   < 5   Neg   None   Res-Rem   17  
9   62   AAF   Idiopathic   Initial   8   0.213   1062   No   150.28   Yes   AMS   < 5   Neg   P, V, S   Res-Rem   8  
10   62   WF   Idiopathic   Multi   12   0.304   1084   No   97.24   Yes   None   < 5   6.5*  CVP, S   Rem-Rel-Rem   35  
11   52   AAF   Idiopathic   Multi   20   0.20   866   No   97.24   No   None   < 5   8*  P, S, CVP   Rem-Rel-Died   2† 
12   44   AAF   Idiopathic   Multi   15   0.23   695   No   97.24   Yes   Stroke, blindness   < 5   10*  CVP, S, CSA, rituximab   Rem-Rel-Rem   48  
13   21   WF   Idiopathic   Initial   9   0.129   1489   No   132.6   No   AMS   < 5   5*  P   Res-Rem   18  
14   26   WF   Idiopathic   Multi   18   0.25   680   No   79.56   Yes   Headache   < 5   1*  P   Rem-Rel-Rem   16  
15   42   WF   Idiopathic   Initial   42   0.249   1584   No   159.12   No   None   25   Neg   P   Res-Rem   32  
16   42   AAF   Idiopathic   Initial   16   0.238   1262   No   150.28   No   None   14   Neg   None   Res-Rem   32  
17   73   AAF   Idiopathic   Multi   35   0.28   939   No   61.88   No   None   < 5   5*  P, S   Rem-Rel-Died   0.03  
18   46   AAF   Idiopathic   Multi   40   0.32   409   No   238.68   No   Aphasia   < 5   Neg   P, V, S   Rem-Rel-Died   0.5  
19   37   WF   Idiopathic   Multi   80   0.37   428   Yes   380.12   No   AMS   46   Neg   None   Rem-Rel-Rem   22  
20   59   WF   Idiopathic   Initial   47   0.247   638   No   265.2   No   None   < 5   1*  None   Res-Rem   7  
21   54   WF   HSCT   Initial   25   0.326   1150   No   44.2   No   AMS   41   Neg   None   NR-Died   0.6  
22   54   WF   HSCT   Initial   11   0.225   463   No   132.6   No   AMS   16   Neg   None   Res-Rem   25  
23   19   WM   HSCT   Initial   34   0.283   328   No   70.72   Yes   None   56   Neg   P   Res-Rem   30  
24   45   AsiF   HSCT   Initial   15   0.296   1483   No   141.44   No   None   66   Neg   None   NR   25  
25   44   WM   HSCT   Initial   8   0.31   1902   Yes   565.76   Yes   Slurred speech   64   Neg   P   NR-Died   1.3  
26   25   WM   HSCT   Initial   13   0.236   1678   No   424.32   No   AMS   84   Neg   None   NR-Died   ?  
27   30   WM   HSCT   Multi   16   0.263   1362   No   291.72   Yes   AMS   32   Neg   P   Rem-Rel-Died   3  
28   47   WF   HSCT   Initial   4   0.261   648   Yes   194.48   No   AMS   32   Neg   P   Res-Rem   35  
29   69   WF   FK506   Initial   23   0.252   832   No   150.28   Yes   None   34   Neg   P   Res-Died   1.3  
30   44   WM   Cancer   Initial   147   0.25   839   No   344.76   No   None   66   Neg   P   NR-Died   2  
31   52   AAF   Cancer   Multi   18   0.24   1477   Yes   238.68   Yes   Syncope   48   Neg   P   Res-Died   5  
32   59   WF   Mitomycin   Initial   2   0.19   2383   Yes   97.24   No   AMS   29   Neg   None   NR-Died   36  
33   74   WM   Clopidogrel   Multi   26   0.25   263   Yes   247.52   Yes   None   96   Neg   P, V   NR-Died   1.6  
34   16   AAM   Lupus   Initial   41   0.238   ND   No   176.8   No   None   52   Neg   P, S, V   NR-Died   2  
35   17   AAF   SCD   Initial   39   0.221   28000   Yes   309.4   No   AMS   74   Neg   None   Res-Rem   19  
36   29   WF   Postpartum   Initial   36   0.28   2401   No   256.36   No   None   48   Neg   None   Res-Rem   32  
37
 		 36
 		 WF
 		 Pregnancy
 		 Initial
 		 46
 		 0.266
 		 1118
 		 No
 		 53.04
 		 Yes
 		 None
 		 116
 		 Neg
 		 None
 		 Res-Rem
 		 28
 

Entries correspond to values on the day of admission. All patients received plasma exchange therapy. For ADAMTS 13 inhibitor, Neg indicates a negative inhibitor screen with 1:1 mixing study as described in “Patients, materials, and methods”; low titer (< 5 units/mL) of inhibitor was detected in patients 14 and 20. High titer (> 5 units/mL) was detected in patients 10, 11, 12, 13, and 17 on admission.

Plt indicates platelet count; Hct, hematocrit; Cr, creatinine; CNS, central nervous system; F/U, the length of follow-up in months since patients were enrolled into the study; AAF, African American female; Res, response; Rem, remission; P, prednisone; V, vincristine; S, splenectomy; WF, white female; Multi, history of multiple episodes of TTP at presentation; AMS, altered mental status; IVIg, intravenous immunoglobulin; CVP, a combination of cyclophosphamide, vincristine, and prednisone; Rel, relapse; CSA, cyclosporine; HSCT, hematopoietic stem cell transplantation; NR, no response; WM, white male; AsiF, Asian female; Lupus, positive antinuclear antibody; ND, not done; ?, serum LDH not done for this patient; and SCD, sickle cell disease.

*

Positive inhibitor identified

†

The time in months between enrollment into the study and patient death

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