Relapses in 301 consecutive patients from the Oklahoma TTP-HUS Registry with a clinical diagnosis of thrombotic thrombocytopenic purpura–hemolytic uremic syndrome (TTP-HUS).*
Clinical Category . | Patients (Total No.) . | Survivors (No.) . | Relapse (No.) . | Comments Regarding Patients 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 | 6 | 0 | |
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 | 0 | |
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%) |
Clinical Category . | Patients (Total No.) . | Survivors (No.) . | Relapse (No.) . | Comments Regarding Patients 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 | 6 | 0 | |
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 | 0 | |
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%) |