Clinical course of the 4 previously published Jehovah’s Witness patients with TTP
| Patient*/year/reference . | Age/sex . | Past medical history, presenting symptoms, laboratory data . | Treatment . | Outcome . |
|---|---|---|---|---|
| 1/2007/5 | 19/F | Previous mixed connective tissue disease treated with hydroxychloroquine and prednisone. Chest pain, headache, syncope. Hgb, 4.9; Plt <10; Cr, 1.0; LDH, 1131; ADAMTS13, <5%; Inh, <0.4 BU | MP, 1000 mg on days 1-3. Vincristine, day 3, 2 mg; days 6 and 9, 1 mg. | Plt, <10 on days 1-3; 125 on day 4; 280 on day 5 |
| 2/2015/6 | 41/F | Previous SLE, treated with hydroxychloroquine. Bruising, dyspnea, abdominal pain. Hgb, 6.1; Plt, 29; Cr, “normal”; LDH, 477; ADAMTS13, <5%; Inh, 1.4 BU | Dex, 40 mg days 1-4, IVIg, 1 g/kg days 1, 2, 5. Vincristine, 2 mg days 3, 8. Rituximab, 375 mg/m2 days 3, 8, 15. Apheresis with albumin replacement days 2, 3, 6, 7. Epo, 30 000 U days 6-11. | Plt, 9-29 until 50 on day 8; 200 on day 12. Nadir Hgb, 3.2 on day 8, 6.3 on day 16. At 6 mo: Hgb, 12.6; Plt, 225; ADAMTS13, 90% |
| 3/2015/8 | 58/F | First episode: Hgb, 5.4; Plt, 16; Cr, 1.2; LDH, 1605; Hpt, <10; ADAMTS13, NR | First episode: rituximab, cyclophosphamide | Both episodes: “Significant improvement within few days” (remission is assumed) |
| Second episode: Transient aphasia, blurred vision. Hgb, 9.1; Plt, 30; Cr, 1.1; LDH, 756; ADAMTS13, NR | Second episode: rituximab, IVIg | |||
| 4/2017/7 | 22/F | Purpura. Hgb, 10.1; Plt, 12; LDH, increased; Hpt, decreased; ADAMTS13, <5%; Inh, >8 BU | “High-dose steroid,” Epo, folic acid beginning day 1. Apheresis, albumin replacement: days 2, 5; cryoprecipitate replacement, days 7, 9, 10, 12, 13. Rituximab, days 3, 10, 17. Vincristine, days 6, 16. Sanguinate, days 10-13. | Discharge on day 19: Hgb, 8.8; Plt, 221 |
| Patient*/year/reference . | Age/sex . | Past medical history, presenting symptoms, laboratory data . | Treatment . | Outcome . |
|---|---|---|---|---|
| 1/2007/5 | 19/F | Previous mixed connective tissue disease treated with hydroxychloroquine and prednisone. Chest pain, headache, syncope. Hgb, 4.9; Plt <10; Cr, 1.0; LDH, 1131; ADAMTS13, <5%; Inh, <0.4 BU | MP, 1000 mg on days 1-3. Vincristine, day 3, 2 mg; days 6 and 9, 1 mg. | Plt, <10 on days 1-3; 125 on day 4; 280 on day 5 |
| 2/2015/6 | 41/F | Previous SLE, treated with hydroxychloroquine. Bruising, dyspnea, abdominal pain. Hgb, 6.1; Plt, 29; Cr, “normal”; LDH, 477; ADAMTS13, <5%; Inh, 1.4 BU | Dex, 40 mg days 1-4, IVIg, 1 g/kg days 1, 2, 5. Vincristine, 2 mg days 3, 8. Rituximab, 375 mg/m2 days 3, 8, 15. Apheresis with albumin replacement days 2, 3, 6, 7. Epo, 30 000 U days 6-11. | Plt, 9-29 until 50 on day 8; 200 on day 12. Nadir Hgb, 3.2 on day 8, 6.3 on day 16. At 6 mo: Hgb, 12.6; Plt, 225; ADAMTS13, 90% |
| 3/2015/8 | 58/F | First episode: Hgb, 5.4; Plt, 16; Cr, 1.2; LDH, 1605; Hpt, <10; ADAMTS13, NR | First episode: rituximab, cyclophosphamide | Both episodes: “Significant improvement within few days” (remission is assumed) |
| Second episode: Transient aphasia, blurred vision. Hgb, 9.1; Plt, 30; Cr, 1.1; LDH, 756; ADAMTS13, NR | Second episode: rituximab, IVIg | |||
| 4/2017/7 | 22/F | Purpura. Hgb, 10.1; Plt, 12; LDH, increased; Hpt, decreased; ADAMTS13, <5%; Inh, >8 BU | “High-dose steroid,” Epo, folic acid beginning day 1. Apheresis, albumin replacement: days 2, 5; cryoprecipitate replacement, days 7, 9, 10, 12, 13. Rituximab, days 3, 10, 17. Vincristine, days 6, 16. Sanguinate, days 10-13. | Discharge on day 19: Hgb, 8.8; Plt, 221 |
Doses, regimens: Cr, mg/dL; Hgb, g/dL; Hpt, mg/dL; LDH, U/L; Plt, × 109/L.
BU, Bethesda unit; Cr, serum creatinine; Dex, dexamethasone; Hpt, haptoglobin; Inh, functional ADAMTS13 inhibitor; LDH, lactate dehydrogenase; MP, methylprednisolone; NR, not reported; SLE, systemic lupus erythematosus.
Patients 1, 2, and 4: platelet count values were extrapolated from the published figures. Patient 3 was described in an abstract with minimal detail. No presenting symptoms were described for her first episode; ADAMTS13 activity was not reported. No regimens or days of administration were reported for the treatments; no outcomes were described for either episode. Patient 4: Sanguinate (pegylated bovine carboxyhemoglobin) is a product in development for patients with severe anemia (NCT02754999). For patients 3 and 4, drug doses were not reported.