Table 1.

Clinical characteristics of iTTP patients at baseline, within 30 days of remission (month 0) and 6 months after remission (month 6)

BaselineMonth 0Month 6
Demographic characteristics (N = 7)*    
 Women, n (%) 5 (71%)   
 Age at symptom onset, y    
  Mean (SD) 48 (19)   
  Median (25th, 75th percentiles) 50 (30, 59)   
  Range 21-77   
 Body mass index, kg/m2    
  Mean (SD) 36 (8)   
  Median (25th, 75th percentiles) 33 (33, 41)   
  Range 25-48   
Clinical characteristics    
 Platelet count*, × 109/L    
  Mean (SD) 22 (25) 251 (41) 239 (44) 
  Median (25th, 75th percentiles) 11 (10, 26) 248 (229, 290) 239 (223, 261) 
  Range <10-76 183-299 161-306 
 Hemoglobin, g/L    
  Mean (SD) 84 (18) 126 (13) 128 (17) 
  Median (25th, 75th percentiles) 79 (78, 102) 126 (122, 141) 121 (116, 149) 
  Range 53-106 102, 142 109 – 153 
 Lactate dehydrogenase, U/L (upper limit of normal, 214 U/L)    
  Mean (SD) 1465 (813) 254 (114) 217 (83) 
  Median (25th, 75th percentiles) 1420 (671, 2256) 249 (161, 339) 197 (143, 263) 
  Range 417-2500 79, 413 134-371 
 ADAMTS13,   
  Mean (SD) <1 61 (41)§ 75 (40)§ 
  Median (25th, 75th percentiles) <1 81 (19, 91) 89 (52, 114) 
  Range <1 <1, 96 <1->114 
 Serum creatinine, µmol/L    
  Mean (SD) 123 (40) 100 (56) 75 (18) 
  Median (25th, 75th percentiles) 122 (95, 157) 73 (66, 107) 71 (64, 76) 
  Range 65-186 58, 220 62-115 
  Missing, n (%) 0 (0%) 0 (0%)  
Immunosuppressive therapy    
 Prednisone, n (%) 7 (100%)   
 Rituximab, n (%) 6 (86%)   
 Vincristineǁ 2 (29%)   
BaselineMonth 0Month 6
Demographic characteristics (N = 7)*    
 Women, n (%) 5 (71%)   
 Age at symptom onset, y    
  Mean (SD) 48 (19)   
  Median (25th, 75th percentiles) 50 (30, 59)   
  Range 21-77   
 Body mass index, kg/m2    
  Mean (SD) 36 (8)   
  Median (25th, 75th percentiles) 33 (33, 41)   
  Range 25-48   
Clinical characteristics    
 Platelet count*, × 109/L    
  Mean (SD) 22 (25) 251 (41) 239 (44) 
  Median (25th, 75th percentiles) 11 (10, 26) 248 (229, 290) 239 (223, 261) 
  Range <10-76 183-299 161-306 
 Hemoglobin, g/L    
  Mean (SD) 84 (18) 126 (13) 128 (17) 
  Median (25th, 75th percentiles) 79 (78, 102) 126 (122, 141) 121 (116, 149) 
  Range 53-106 102, 142 109 – 153 
 Lactate dehydrogenase, U/L (upper limit of normal, 214 U/L)    
  Mean (SD) 1465 (813) 254 (114) 217 (83) 
  Median (25th, 75th percentiles) 1420 (671, 2256) 249 (161, 339) 197 (143, 263) 
  Range 417-2500 79, 413 134-371 
 ADAMTS13,   
  Mean (SD) <1 61 (41)§ 75 (40)§ 
  Median (25th, 75th percentiles) <1 81 (19, 91) 89 (52, 114) 
  Range <1 <1, 96 <1->114 
 Serum creatinine, µmol/L    
  Mean (SD) 123 (40) 100 (56) 75 (18) 
  Median (25th, 75th percentiles) 122 (95, 157) 73 (66, 107) 71 (64, 76) 
  Range 65-186 58, 220 62-115 
  Missing, n (%) 0 (0%) 0 (0%)  
Immunosuppressive therapy    
 Prednisone, n (%) 7 (100%)   
 Rituximab, n (%) 6 (86%)   
 Vincristineǁ 2 (29%)   
*

There are no missing values for these demographic and clinical characteristics.

Platelet count < 10 × 109/L (our laboratory does not quantify < 10 × 109/L); a value of 5 × 109/L was given for this summary.

All patients had ADAMTS13 levels < 10% and inhibitor levels > 14 U/mL. All patients had detectable inhibitor level.

§

A value of <1% was replaced with a value of 1 for this summary; a value of >114 was replaced with 114 for this summary. Inhibitors were not run because most patients had normalized their ADAMTS13 activity.

ǁ

Used in cases of refractory iTTP not responsive to TPE and corticosteroids alone.

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