Discrepant activity levels of von willebrand factor-cleaving protease (ADAMTS-13) in congenital thrombotic thrombocytopenic purpura [6]

Blood

Volumn 102, Issue 3, 2003, Pages 1148-

  Studt, Jan Dirk ^b   Hovinga, Johanna A Kremer ^b   Furlan, Miha ^b   Lämmle, Bernhard ^a  

^a UNIVERSITY OF BERN   (Switzerland)

^b NONE

Author keywords

[No Author keywords available]

Indexed keywords

FRESH FROZEN PLASMA; PROTEINASE; UNCLASSIFIED DRUG; VON WILLEBRAND FACTOR; VON WILLEBRAND FACTOR CLEAVING PROTEINASE;

ENZYME ACTIVITY; ENZYME DEFICIENCY; GENE MUTATION; HUMAN; IMMUNOBLOTTING; LETTER; MICROANGIOPATHY; PLASMA TRANSFUSION; PRIORITY JOURNAL; THROMBOTIC THROMBOCYTOPENIC PURPURA;

EID: 0042243489     PISSN: 00064971     EISSN: None     Source Type: Journal    
DOI: 10.1182/blood-2003-05-1466     Document Type: Letter

References (7)

1. Savasan S, Lee SK, Ginsburg D, Tsai HM. ADAMTS13 gene mutation in congenital thrombotic thrombocytopenic purpura with previously reported normal VWF cleaving protease activity. Blood. 2003;101:4449-4451.
2. Savasan S, Taub JW, Buck S, Botterill M, Furlan M, Ravindranath Y. Congenital microangiopathic hemolytic anemia and thrombocytopenia with unusually large von Willebrand factor multimers and von Willebrand factor-cleaving protease. J Pediatr Hematol Oncol. 2001;23:364-367.
3. Furlan M, Robles R, Solenthaler M, Wassmer M, Sandoz P, Lämmle B. Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura. Blood. 1997;89:3097-3103.
4. Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med. 1998;339:1585-1594.
5. Furlan M, Robles R, Galbusera M, et al. Von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med. 1998;339:1578-1584.
6. Studt JD, Böhm M, Budde U, Girma JP, Varadi K, Lämmle B. Measurement of von Willebrand factor-cleaving protease (ADAMTS-13) activity in plasma: a multicenter comparison of different assay methods. J Thromb Haemost. In press.
7. Furlan M, Robles R, Morselli B, Sandoz P, Lämmle B. Recovery and half-life of von Willebrand factor-cleaving protease after plasma therapy in patients with thrombotic thrombocytopenic purpura. Thromb Haemost. 1999;81:8-13.