Table 2

Clinical categories TTP and frequency of severe ADAMTS13 deficiency: data from all patients who had ADAMTS13 measurements (1995-2009)

CategoryAll patients (n = 283)Patients with ADAMTS13 < 10% (n = 65)
Allogeneic HSCT 10 1 (10%) 
Pregnant/postpartum 15 3 (21%) 
Drug-associated (n = 38)   
    Quinine 20 
    Chemotherapeutic agents, calcineurin inhibitor 12 
    Other drugs 
Bloody diarrhea 25 2 (8%) 
Additional/alternative disorders (n = 88)   
    SLE 21 2 (10%) 
    Other autoimmune disorders 16 1 (7%) 
    Infections 23 4 (17%) 
    Malignancies 10 1 (10%) 
    Malignant hypertension 
    Other disorders 12 
Idiopathic 107 51 (48%) 
CategoryAll patients (n = 283)Patients with ADAMTS13 < 10% (n = 65)
Allogeneic HSCT 10 1 (10%) 
Pregnant/postpartum 15 3 (21%) 
Drug-associated (n = 38)   
    Quinine 20 
    Chemotherapeutic agents, calcineurin inhibitor 12 
    Other drugs 
Bloody diarrhea 25 2 (8%) 
Additional/alternative disorders (n = 88)   
    SLE 21 2 (10%) 
    Other autoimmune disorders 16 1 (7%) 
    Infections 23 4 (17%) 
    Malignancies 10 1 (10%) 
    Malignant hypertension 
    Other disorders 12 
Idiopathic 107 51 (48%) 

Data are presented for 283 consecutive patients in whom ADAMTS13 was measured immediately before their first plasma exchange treatment (November 13, 1995 through December 31, 2009). Patients were assigned to clinical categories based on their clinical features during their initial episode and without knowledge of the results of the ADAMTS13 measurement, as previously described.17  Chemotherapeutic agents were mitomycin C (4), gemcitabine (4), carmustine (1), and pentostatin (1). The calcineurin inhibitor was cyclosporine (2). Other drugs were alendronate, clopidogrel, cocaine, ticlopidine, trimethoprim-sulfamethoxazole, and vancomycin (1 each). Other autoimmune disorders included immune thrombocytopenic purpura/Evan syndrome (5), antiphospholipid antibody syndrome (2), scleroderma (2), Wegener granulomatosis (2), and once each for bronchiolitis obliterans with organizing pneumonia, Hamman-Rich syndrome, periarteritis nodosa, Sjögren syndrome, and unspecified vasculitis. Other disorders included multiorgan failure (11) and congenital hemolytic anemia (1).