TY - JOUR
T1 - Does cryosupernatant plasma improve outcome in thrombotic thrombocytopenic purpura? No answer yet
AU - Rock, Gail
AU - Anderson, David
AU - Clark, William
AU - Leblond, Pierre
AU - Palmer, Douglas
AU - Sternbach, Marion
AU - Sutton, David
AU - Wells, George
AU - Benny, Barret
AU - Buskard, Noel
AU - Devaraj, Ramachand
AU - Dolan, S.
AU - Freedman, John
AU - Klassen, John
AU - Kovithavongs, T.
AU - Sheridan, David
AU - Shore, Sappora
AU - Shumak, Kenneth
AU - Vickers, Linda
PY - 2005/4
Y1 - 2005/4
N2 - A randomized prospective trial compared cryosupernatant plasma (CSP) to fresh frozen plasma (FFP) for treatment of thrombotic thrombocytopenic purpura (TTP). A total of 236 patients were required: 28 patients were treated with CSP and 24 with FFP within 30 months. There were no differences in survival at 1 month. By day 9, 17 of 26 patients with CSP and 18 of 24 with FFP had a platelet count >100 × 109/l. At entry, von Willebrand factor (VWF) multimers were normal in all patients (range 1·1-3·95 IU/ml). ADAMTS-13 levels showed large variations ranging from 10% to 100% activity. At entry, no individual had <5% VWF cleaving protease. By day 9 (end of cycle), 89% (FFP) and 67% (CSP) had levels >50% of the controls. At 6 months some patients showed inhibitors to the enzyme in spite of adequate or normal platelet counts. The data from this study do not show an apparent advantage to the use of CSP in TTP. A large number of patients will be required to determine appropriate replacement therapy. We were not able to find a statistically significant relationship between the low level of protease activity at presentation of TTP and response.
AB - A randomized prospective trial compared cryosupernatant plasma (CSP) to fresh frozen plasma (FFP) for treatment of thrombotic thrombocytopenic purpura (TTP). A total of 236 patients were required: 28 patients were treated with CSP and 24 with FFP within 30 months. There were no differences in survival at 1 month. By day 9, 17 of 26 patients with CSP and 18 of 24 with FFP had a platelet count >100 × 109/l. At entry, von Willebrand factor (VWF) multimers were normal in all patients (range 1·1-3·95 IU/ml). ADAMTS-13 levels showed large variations ranging from 10% to 100% activity. At entry, no individual had <5% VWF cleaving protease. By day 9 (end of cycle), 89% (FFP) and 67% (CSP) had levels >50% of the controls. At 6 months some patients showed inhibitors to the enzyme in spite of adequate or normal platelet counts. The data from this study do not show an apparent advantage to the use of CSP in TTP. A large number of patients will be required to determine appropriate replacement therapy. We were not able to find a statistically significant relationship between the low level of protease activity at presentation of TTP and response.
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U2 - 10.1111/j.1365-2141.2005.05418.x
DO - 10.1111/j.1365-2141.2005.05418.x
M3 - Article
C2 - 15801959
AN - SCOPUS:20244390173
SN - 0007-1048
VL - 129
SP - 79
EP - 86
JO - British Journal of Haematology
JF - British Journal of Haematology
IS - 1
ER -