Risks for death and relapse in thrombotic thrombocytopenic purpura (TTP) related to ADAMTS13 deficiency.*
| . | ADAMTS13 Deficient . | ADAMTS13 Not Deficient . | ||||
|---|---|---|---|---|---|---|
| Case Series (no. of patients) . | Total No. Patients . | Death (no.) . | Relapse (no.) . | Total No. Patients . | Death (no.) . | Relapse (no.) . |
| * Data from 5 case series describing the occurrence of death and relapse among patients distinguished by severe deficiency or lack of severe deficiency of ADAMTS13 activity. | ||||||
| Data from Veyradier et al15 presented here are on 63 of 111 patients who were described as having TTP rather than hemolytic uremic syndrome (HUS). Deaths were not reported. Patients were distinguished as “sporadic” or “intermittent.” For this table, sporadic was assumed to be a single episode while intermittent was assumed to indicate the occurrence of relapses. | ||||||
| Data from Mori et al12 presented here are on 18 of 27 patients who were described as having TTP rather than HUS. Relapses were not reported. | ||||||
| Data from Raife et al14 presented here are on 107 consecutive patients described as having thrombotic microangiopathy; syndromes resembling TTP or HUS were not distinguished. Relapses were not reported. | ||||||
| All 37 patients in the study of Zheng et al13 were described as having TTP. Patients who were described as idiopathic had no apparent pre-existing illness. Patients described as non-idiopathic had had hematopoietic stem cell transplantation, were pregnant or postpartum, had systemic lupus erythematosis, or had taken FK506, mitomycin C, or clopidogrel. | ||||||
| Data from Vesely et al3 presented here are on 185 patients who had ADAMTS13 measurements at the time of their initial presentation, representing 90% of the 206 patients enrolled in the Oklahoma TTP-HUS Registry, November 13, 1995-December 31, 2003. Data on 142 of these patients, up to June 30, 2002, have been previously published.3 Patients defined as idiopathic had no apparent etiology or associated clinical conditions. Patients described as non-idiopathic had had hematopoietic stem cell transplantation, were pregnant or postpartum, had taken a drug associated with TTP-HUS, had a bloody diarrhea prodrome, or had an additional/alternative diagnosis. Deaths are reported if they occurred within 30 days of stopping plasma exchange treatment. Relapses are reported in patients who survived for more than 30 days following their plasma exchange treatment. | ||||||
| Veyradier et al15 (63) | 44 | – | 10 | 19 | – | 0 |
| Mori et al12 (18) | 12 | 2 | – | 6 | 4 | – |
| Raife et al14 (107) | 50 | 4 | – | 57 | 10 | – |
| Zheng et al13 idiopathic (20) non-idiopathic (17) | 16 0 | 3 – | 6 – | 4 17 | 0 10 | 1 1 |
| Vesely et al3 idiopathic (67) non-idiopathic (118) | 20 2 | 4 0 | 7/16 1/2 | 47 116 | 7 48 | 3/40 2/68 |
| . | ADAMTS13 Deficient . | ADAMTS13 Not Deficient . | ||||
|---|---|---|---|---|---|---|
| Case Series (no. of patients) . | Total No. Patients . | Death (no.) . | Relapse (no.) . | Total No. Patients . | Death (no.) . | Relapse (no.) . |
| * Data from 5 case series describing the occurrence of death and relapse among patients distinguished by severe deficiency or lack of severe deficiency of ADAMTS13 activity. | ||||||
| Data from Veyradier et al15 presented here are on 63 of 111 patients who were described as having TTP rather than hemolytic uremic syndrome (HUS). Deaths were not reported. Patients were distinguished as “sporadic” or “intermittent.” For this table, sporadic was assumed to be a single episode while intermittent was assumed to indicate the occurrence of relapses. | ||||||
| Data from Mori et al12 presented here are on 18 of 27 patients who were described as having TTP rather than HUS. Relapses were not reported. | ||||||
| Data from Raife et al14 presented here are on 107 consecutive patients described as having thrombotic microangiopathy; syndromes resembling TTP or HUS were not distinguished. Relapses were not reported. | ||||||
| All 37 patients in the study of Zheng et al13 were described as having TTP. Patients who were described as idiopathic had no apparent pre-existing illness. Patients described as non-idiopathic had had hematopoietic stem cell transplantation, were pregnant or postpartum, had systemic lupus erythematosis, or had taken FK506, mitomycin C, or clopidogrel. | ||||||
| Data from Vesely et al3 presented here are on 185 patients who had ADAMTS13 measurements at the time of their initial presentation, representing 90% of the 206 patients enrolled in the Oklahoma TTP-HUS Registry, November 13, 1995-December 31, 2003. Data on 142 of these patients, up to June 30, 2002, have been previously published.3 Patients defined as idiopathic had no apparent etiology or associated clinical conditions. Patients described as non-idiopathic had had hematopoietic stem cell transplantation, were pregnant or postpartum, had taken a drug associated with TTP-HUS, had a bloody diarrhea prodrome, or had an additional/alternative diagnosis. Deaths are reported if they occurred within 30 days of stopping plasma exchange treatment. Relapses are reported in patients who survived for more than 30 days following their plasma exchange treatment. | ||||||
| Veyradier et al15 (63) | 44 | – | 10 | 19 | – | 0 |
| Mori et al12 (18) | 12 | 2 | – | 6 | 4 | – |
| Raife et al14 (107) | 50 | 4 | – | 57 | 10 | – |
| Zheng et al13 idiopathic (20) non-idiopathic (17) | 16 0 | 3 – | 6 – | 4 17 | 0 10 | 1 1 |
| Vesely et al3 idiopathic (67) non-idiopathic (118) | 20 2 | 4 0 | 7/16 1/2 | 47 116 | 7 48 | 3/40 2/68 |