Table 1.

Demographic, clinical, and treatment data for the patient cohort

Entire cohort (N = 124)RTX (N = 60)No RTX (N = 64)
Study site, n (%)    
 Harvard Consortium 91 (73) 46 (77) 45 (70) 
 University of Washington 17 (14) 9 (15) 8 (13) 
 Boston Medical Center 16 (13) 5 (8) 11 (17) 
Type of presentation, n (%)    
 First-time 104 (84) 50 (83) 54 (84) 
 Presenting in relapse 20 (16) 10 (17) 10 (16) 
Sex, n (%)    
 Male 38 (31) 18 (30) 20 (31) 
 Female 86 (69) 42 (70) 44 (69) 
Age, median (IQR), y 42 (31-52) 41 (31-52) 43 (31-53) 
ABO blood group, n (%)    
 Group O 64 (52) 31 (52) 33 (52) 
 Non-group O 58 (47) 27 (45) 31 (48) 
 Unknown 2 (1) 2 (3) 0 (0) 
Ethnicity, n (%)    
 White 67 (54) 34 (57) 33 (52) 
 Nonwhite 52 (42) 24 (40) 28 (44) 
 Unknown 5 (4) 2 (3) 3 (5) 
Laboratory values, median (IQR)    
 ADAMTS13 activity, %* 0 (0-0) 0 (0-0) 0 (0-0) 
 ADAMTS13 inhibitor, BU 1.4 (0.7-2.0) 1.4 (0.8-2.0) 1.4 (0.6-2.0) 
 Hemoglobin, g/dL 8.9 (7.3-10.1) 8.6 (7.1-9.6) 9.4 (7.4-10.8) 
 Platelets, ×109/L 16 (10-22) 15 (10-20) 17 (11-24) 
 Lactate dehydrogenase, U/L 1090 (742-1402) 1089 (734-1504) 1090 (782-1329) 
 Cr, mg/dL 1.0 (0.8-1.4) 1.0 (0.7-1.3) 1.1 (0.9-1.6) 
 Total bilirubin, mg/dL 2.3 (1.5-3.1) 2.4 (1.5-3.1) 2.3 (1.5-3.1) 
 Reticulocyte, % 4.6 (2.9-8.3) 6.2 (3.7-9.9) 3.6 (2.6-6.4) 
 Day 4 platelets, ×109/L 131 (68-181) 111 (49-160) 159 (107-216) 
 Days to platelet normalization 5 (4-8) 6 (4-15.3) 4 (3.5-6) 
Treatment    
 No. (%) treated with TPE 120 (97) 60 (100) 60 (94) 
  Median (IQR) TPE procedures 15 (8-23) 22 (14-29) 9 (6-15) 
 No. (%) treated with steroids 113 (91) 58 (97) 56 (88) 
 No. (%) treated with second-line drug 8 (6.5) 8 (13) 0 (0) 
 Median (IQR) hospital stay, d 12 (8-20) 18 (11-27) 9 (7-14) 
Entire cohort (N = 124)RTX (N = 60)No RTX (N = 64)
Study site, n (%)    
 Harvard Consortium 91 (73) 46 (77) 45 (70) 
 University of Washington 17 (14) 9 (15) 8 (13) 
 Boston Medical Center 16 (13) 5 (8) 11 (17) 
Type of presentation, n (%)    
 First-time 104 (84) 50 (83) 54 (84) 
 Presenting in relapse 20 (16) 10 (17) 10 (16) 
Sex, n (%)    
 Male 38 (31) 18 (30) 20 (31) 
 Female 86 (69) 42 (70) 44 (69) 
Age, median (IQR), y 42 (31-52) 41 (31-52) 43 (31-53) 
ABO blood group, n (%)    
 Group O 64 (52) 31 (52) 33 (52) 
 Non-group O 58 (47) 27 (45) 31 (48) 
 Unknown 2 (1) 2 (3) 0 (0) 
Ethnicity, n (%)    
 White 67 (54) 34 (57) 33 (52) 
 Nonwhite 52 (42) 24 (40) 28 (44) 
 Unknown 5 (4) 2 (3) 3 (5) 
Laboratory values, median (IQR)    
 ADAMTS13 activity, %* 0 (0-0) 0 (0-0) 0 (0-0) 
 ADAMTS13 inhibitor, BU 1.4 (0.7-2.0) 1.4 (0.8-2.0) 1.4 (0.6-2.0) 
 Hemoglobin, g/dL 8.9 (7.3-10.1) 8.6 (7.1-9.6) 9.4 (7.4-10.8) 
 Platelets, ×109/L 16 (10-22) 15 (10-20) 17 (11-24) 
 Lactate dehydrogenase, U/L 1090 (742-1402) 1089 (734-1504) 1090 (782-1329) 
 Cr, mg/dL 1.0 (0.8-1.4) 1.0 (0.7-1.3) 1.1 (0.9-1.6) 
 Total bilirubin, mg/dL 2.3 (1.5-3.1) 2.4 (1.5-3.1) 2.3 (1.5-3.1) 
 Reticulocyte, % 4.6 (2.9-8.3) 6.2 (3.7-9.9) 3.6 (2.6-6.4) 
 Day 4 platelets, ×109/L 131 (68-181) 111 (49-160) 159 (107-216) 
 Days to platelet normalization 5 (4-8) 6 (4-15.3) 4 (3.5-6) 
Treatment    
 No. (%) treated with TPE 120 (97) 60 (100) 60 (94) 
  Median (IQR) TPE procedures 15 (8-23) 22 (14-29) 9 (6-15) 
 No. (%) treated with steroids 113 (91) 58 (97) 56 (88) 
 No. (%) treated with second-line drug 8 (6.5) 8 (13) 0 (0) 
 Median (IQR) hospital stay, d 12 (8-20) 18 (11-27) 9 (7-14) 
*

An undetectable ADAMTS13 activity level was recorded as 0%.

Five patients were given cyclophosphamide, 2 were given bortezomib (1 in combination with tacrolimus), and 1 patient was given vincristine.

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