Table 1.

Clinical features and treatment of patients according to relapse status after acute presentation

Clinical featureNo relapse (n = 312, 70.4%)Relapse (n = 131, 29.6%)P
Duration of follow-up (IQR), y 8.4 (5.7-10.4) 8.7 (5.6-10.9) .39 
Age at presentation, y 45.0 45.1 .49 
Age <25 y at presentation, n (%) 27 (8.7) 11 (8.4) .93 
Gender (male/female), n (%) 79 (25.3)/233 (74.7) 43 (32.8)/88 (67.2) .11 
Blood counts at presentation    
Hemoglobin, g/L 82 95 <.001 
Platelets, ×109/L 20 34 <.001 
Absolute reticulocytes, ×109/L 158 159 .95 
Ethnicity, n (%)   .019 
Caucasian 197 (63.1) 69 (52.7)  
Black African 32 (10.2) 13 (9.9)  
Black Caribbean 22 (7.1) 22 (16.8)  
Asian 26 (8.3) 14 (10.7)  
Mixed 5 (1.6) 4 (3.1)  
Other 6 (1.9) 3 (2.3)  
Unknown 24 (7.7) 6 (4.6)  
Associated conditions, n (%)   <.001 
Idiopathic 202 (64.7) 87 (66.4)  
HIV 17 (5.4) 3 (2.3)  
Drug-induced 5 (1.6) 0 (0)  
Other autoimmune disease 51 (16.3) 27 (20.6)  
Pancreatitis 11 (3.5) 0 (0)  
Pregnancy 10 (3.2) 8 (6.1)  
Other 16 (5.1) 7 (5.3)  
Treatment at acute presentation, n (%)    
Corticosteroid 267 (85.6) 116 (88.5) .97 
Rituximab 169 (54.2)   
Mycophenolate mofetil 2 (0.6)  
Caplacizumab 7 (2.2) 3 (2.3)  
Clinical featureNo relapse (n = 312, 70.4%)Relapse (n = 131, 29.6%)P
Duration of follow-up (IQR), y 8.4 (5.7-10.4) 8.7 (5.6-10.9) .39 
Age at presentation, y 45.0 45.1 .49 
Age <25 y at presentation, n (%) 27 (8.7) 11 (8.4) .93 
Gender (male/female), n (%) 79 (25.3)/233 (74.7) 43 (32.8)/88 (67.2) .11 
Blood counts at presentation    
Hemoglobin, g/L 82 95 <.001 
Platelets, ×109/L 20 34 <.001 
Absolute reticulocytes, ×109/L 158 159 .95 
Ethnicity, n (%)   .019 
Caucasian 197 (63.1) 69 (52.7)  
Black African 32 (10.2) 13 (9.9)  
Black Caribbean 22 (7.1) 22 (16.8)  
Asian 26 (8.3) 14 (10.7)  
Mixed 5 (1.6) 4 (3.1)  
Other 6 (1.9) 3 (2.3)  
Unknown 24 (7.7) 6 (4.6)  
Associated conditions, n (%)   <.001 
Idiopathic 202 (64.7) 87 (66.4)  
HIV 17 (5.4) 3 (2.3)  
Drug-induced 5 (1.6) 0 (0)  
Other autoimmune disease 51 (16.3) 27 (20.6)  
Pancreatitis 11 (3.5) 0 (0)  
Pregnancy 10 (3.2) 8 (6.1)  
Other 16 (5.1) 7 (5.3)  
Treatment at acute presentation, n (%)    
Corticosteroid 267 (85.6) 116 (88.5) .97 
Rituximab 169 (54.2)   
Mycophenolate mofetil 2 (0.6)  
Caplacizumab 7 (2.2) 3 (2.3)  
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