Clinical course of the 5 patients who had ADAMTS13 10% or more at the time of their initial diagnosis with TTP and who were diagnosed with a relapse of TTP
| Patient . | Clinical course . |
|---|---|
| 1 | 64-year-old WF (drug [quinine]–associated category), ADAMTS13 56% (F), 50% (IB). 2nd episode caused by repeat quinine ingestion. ADAMTS13 not measured. |
| 2 | 17-year-old WF (additional or alternative category [autoimmune]) SLE for 5 years; treatment for 2 years. Initial episode: TTP vs SLE flare. Renal biopsy: thrombotic microangiopathy, class IV lupus nephritis. ADAMTS13 70% (F), 80% (IB). 2nd episode: TTP vs SLE flare, renal failure, malignant hypertension. ADAMTS13 > 100% (F, IB). |
| 3 | 17-year-old WF (additional or alternative category [autoimmune]) SLE for 3 months; renal biopsy: class IV lupus nephritis; immunosuppressive treatment. Initial episode: TTP vs SLE flare, renal failure, malignant hypertension. ADAMTS13 40% (F), 20% (IB). 2nd episode: Klebsiella, cytomegalovirus sepsis. ADAMTS13 62% (F), 65% (IB). |
| 4 | 41-year-old BM (idiopathic category), incidental discovery of HIV infection, no signs of immunodeficiency. 1st episode: ADAMTS13 53% (F), 60% (IB). 2nd: not measured. 3rd: 15% (F), 50% (IB), INH 1.4 BU. 4th: < 5% (F), 8% (IB), 0.8 BU. 5th and 6th: < 5% (F, IB), INH 1.1 and 1.4 BU.29,30 |
| 5 | 30-year-old WF (idiopathic category). Initial episode, ADAMTS13 16% (F), 100% (IB), INH 1.9 BU. 2nd episode, 29% (F), 100% (IB), INH 1.3 BU. |
| Patient . | Clinical course . |
|---|---|
| 1 | 64-year-old WF (drug [quinine]–associated category), ADAMTS13 56% (F), 50% (IB). 2nd episode caused by repeat quinine ingestion. ADAMTS13 not measured. |
| 2 | 17-year-old WF (additional or alternative category [autoimmune]) SLE for 5 years; treatment for 2 years. Initial episode: TTP vs SLE flare. Renal biopsy: thrombotic microangiopathy, class IV lupus nephritis. ADAMTS13 70% (F), 80% (IB). 2nd episode: TTP vs SLE flare, renal failure, malignant hypertension. ADAMTS13 > 100% (F, IB). |
| 3 | 17-year-old WF (additional or alternative category [autoimmune]) SLE for 3 months; renal biopsy: class IV lupus nephritis; immunosuppressive treatment. Initial episode: TTP vs SLE flare, renal failure, malignant hypertension. ADAMTS13 40% (F), 20% (IB). 2nd episode: Klebsiella, cytomegalovirus sepsis. ADAMTS13 62% (F), 65% (IB). |
| 4 | 41-year-old BM (idiopathic category), incidental discovery of HIV infection, no signs of immunodeficiency. 1st episode: ADAMTS13 53% (F), 60% (IB). 2nd: not measured. 3rd: 15% (F), 50% (IB), INH 1.4 BU. 4th: < 5% (F), 8% (IB), 0.8 BU. 5th and 6th: < 5% (F, IB), INH 1.1 and 1.4 BU.29,30 |
| 5 | 30-year-old WF (idiopathic category). Initial episode, ADAMTS13 16% (F), 100% (IB), INH 1.9 BU. 2nd episode, 29% (F), 100% (IB), INH 1.3 BU. |
Clinical features are described for the 5 patients whose ADAMTS13 activity was ≥ 10% at the time of their initial diagnosis of TTP and who were also diagnosed as having a relapse of TTP. Clinical categories are indicated in parentheses. ADAMTS13 measurements: F, FRETS-VWF73 assay; IB, immunoblotting assay. Citations are provided when patients have been previously described.
SLE indicates systemic lupus erythematosus; TTP, thrombotic thrombocytopenic purpura; INH, inhibitor titer; WF, white female; and BM, black male.