Table 5.

Relapses in 301 consecutive patients from the Oklahoma TTP-HUS Registry with a clinical diagnosis of thrombotic thrombocytopenic purpura–hemolytic uremic syndrome (TTP-HUS).*

ClinicalCategoryPatients(Total No.)Survivors(No.)Relapse(No.)Comments RegardingPatients with Relapses
* Data are presented on all 301 consecutive patients in the Oklahoma TTP-HUS Registry, 1989–2003. The total number of patients in each clinical category is presented; definitions of the clinical categories have been previously published.3 Survival is defined as more than 30 days after the last plasma exchange treatment. Relapse is defined as a diagnosis of TTP-HUS in a patient who has survived more than 30 days following their last plasma exchange. 
Stem cell transplantation 23  
Pregnant/postpartum 25 23 3 (13%) 1 patient, recurrent fetal loss, ? TTP.
 1 patient, 2 relapses, 1 not with pregnancy.
 1 patient, 4 relapses, none with pregnancy,
 initial ADAMTS13 < 5%. 
Drug-association:
 immune-mediated
 dose-dependent 19
 17 15
 14 2 (13%)
 0 Recurrent quinine use 
Bloody diarrhea prodrome 19 13  
Additional/alternative diagnosis 79 34 2 (6%) 1 patient, scleroderma, 3 relapses, ADAMTS 13%–40%.
 1 patient, HIV infection, 4 relapses, initial ADAMTS13
 60%, 5th episode < 3%. 
Idiopathic 119 96 19 (20%) 11 patients, 1 relapse;
 6 patients, 2 relapses;
 2 patients, 4 & 5 relapses
 ADAMTS13 assay on 12 patients; < 5% at initial or
 subsequent episode in 11 (92%) 
ClinicalCategoryPatients(Total No.)Survivors(No.)Relapse(No.)Comments RegardingPatients with Relapses
* Data are presented on all 301 consecutive patients in the Oklahoma TTP-HUS Registry, 1989–2003. The total number of patients in each clinical category is presented; definitions of the clinical categories have been previously published.3 Survival is defined as more than 30 days after the last plasma exchange treatment. Relapse is defined as a diagnosis of TTP-HUS in a patient who has survived more than 30 days following their last plasma exchange. 
Stem cell transplantation 23  
Pregnant/postpartum 25 23 3 (13%) 1 patient, recurrent fetal loss, ? TTP.
 1 patient, 2 relapses, 1 not with pregnancy.
 1 patient, 4 relapses, none with pregnancy,
 initial ADAMTS13 < 5%. 
Drug-association:
 immune-mediated
 dose-dependent 19
 17 15
 14 2 (13%)
 0 Recurrent quinine use 
Bloody diarrhea prodrome 19 13  
Additional/alternative diagnosis 79 34 2 (6%) 1 patient, scleroderma, 3 relapses, ADAMTS 13%–40%.
 1 patient, HIV infection, 4 relapses, initial ADAMTS13
 60%, 5th episode < 3%. 
Idiopathic 119 96 19 (20%) 11 patients, 1 relapse;
 6 patients, 2 relapses;
 2 patients, 4 & 5 relapses
 ADAMTS13 assay on 12 patients; < 5% at initial or
 subsequent episode in 11 (92%) 
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