Does cryosupernatant plasma improve outcome in thrombotic thrombocytopenic purpura? No answer yet.
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 x 10(9)/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.
Rock
Gail
G
Division of Hematology and Transfusion Medicine, Ottawa Civic Hospital, University of Ottawa, Ottawa, ON, Canada. cag@ca.inter.net
Anderson
David
D
Clark
William
W
Leblond
Pierre
P
Palmer
Douglas
D
Sternbach
Marion
M
Sutton
David
D
Wells
George
G
D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; D001323:Autoantibodies / Q000097:blood; D018955:CD36 Antigens / Q000276:immunology; D015925:Cryopreservation; D005169:Factor VIII / Q000378:metabolism; D005260:Female; D006801:Humans; D008666:Metalloendopeptidases / Q000097:blood; D010951:Plasma Exchange / Q000379:methods*; D010976:Platelet Count; D011446:Prospective Studies; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood / Q000628:therapy*; D016019:Survival Analysis; D016896:Treatment Outcome; D014841:von Willebrand Factor / Q000378:metabolism
D001323:Autoantibodies; D018955:CD36 Antigens; D014841:von Willebrand Factor; D005169:Factor VIII; D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human