Von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome

New England Journal of Medicine

Volumn 339, Issue 22, 1998, Pages 1578-1584

  Furlan, Miha ^a   Robles, Rodolfo ^a   Galbusera, Miriam ^b   Remuzzi, Giuseppe ^b   Kyrle, Paul A ^c   Brenner, Brigitte ^c   Krause, Manuela ^d   Scharrer, Inge ^d   Aumann, Volker ^e   Mittler, Uwe ^e   Solenthaler, Max ^a   Lämmle, Bernhard ^a  

^a UNIVERSITY OF BERN   (Switzerland)

^b MARIO NEGRI INST PHARMACOL RES   (Italy)

^c MEDICAL UNIVERSITY OF VIENNA   (Austria)

^d UNIVERSITY HOSPITAL FRANKFURT   (Germany)

^e Department of Perinatology   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

PROTEINASE;

ADULT; ARTICLE; ENZYME ACTIVITY; ENZYME ANALYSIS; ENZYME DEFICIENCY; FEMALE; HEMOLYTIC UREMIC SYNDROME; HUMAN; MAJOR CLINICAL STUDY; MALE; PRIORITY JOURNAL; THROMBOTIC THROMBOCYTOPENIC PURPURA;

ADAM PROTEINS; ADULT; CHILD; FEMALE; HEMOLYTIC-UREMIC SYNDROME; HUMANS; MALE; METALLOENDOPEPTIDASES; PURPURA, THROMBOTIC THROMBOCYTOPENIC; RETROSPECTIVE STUDIES; VON WILLEBRAND FACTOR;

EID: 0032569884     PISSN: 00284793     EISSN: None     Source Type: Journal    
DOI: 10.1056/NEJM199811263392202     Document Type: Article

References (23)

1. Moschcowitz E. Hyaline thrombosis of the terminal arterioles and capillaries: a hitherto undescribed disease. Proc N Y Pathol Soc 1924;24:21-4.
2. Gasser C, Gautier E, Steck A, Siebenmann RE, Oechslin R. Hämolytisch-urämische Syndrome: bilaterale Nierenrindennekrosen bei akuten erworbenen hämolytischen Anämien. Schweiz Med Wochenschr 1955;85:905-9.
3. Moake JL. Haemolytic-uraemic syndrome: basic science. Lancet 1994; 343:393-7.
4. Ruggenenti P, Remuzzi G. The pathophysiology and management of thrombotic thrombocytopenic purpura. Eur J Haematol 1996;56:191-207.
5. Moake JL, Rudy CK, Troll JH, et al. Unusually large plasma factor VIII:von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura. N Engl J Med 1982;307:1432-5.
6. Moake JL, Byrnes JJ, Troll JH, et al. Abnormal VIII: von Willebrand factor patterns in the plasma of patients with the hemolytic-uremic syndrome. Blood 1984;64:592-8.
7. Moake JL, Turner NA, Stathopoulos NA, Nolasco LH, Hellums JD. Involvement of large plasma von Willebrand factor (vWF) multimers and unusually large vWF forms derived from endothelial cells in shear stress-induced platelet aggregation. J Clin Invest 1986;78:1456-61.
8. Chow TW, Turner NA, Chintagumpala M, et al. Increased von Willebrand factor binding to platelets in single episode and recurrent types of thrombotic thrombocytopenic purpura. Am J Hematol 1998;57:293-302.
9. Furlan M, Robles R, Lämmle B. Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood 1996;87:4223-34.
10. Tsai H-M. Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. Blood 1996;87:4235-44.
11. 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;89:3097-103.
12. Furlan M, Robles R, Solenthaler M, Lämmle B. Acquired deficiency of von Willebrand factor-cleaving protease in a patient with thrombotic thrombocytopenic purpura. Blood 1998;91:2839-46.
13. Charba D, Moake JL, Harris MA, Hester JP. Abnormalities of von Willebrand factor multimers in drug-associated thrombotic microangiopathies. Am J Hematol 1993;42:268-77.
14. Frangos JA, Moake JL, Nolasco L, Phillips MD, McIntire LV. Cryosupernatant regulates accumulation of unusually large vWF multimers from endothelial cells. Am J Physiol 1989;256:H1635-H1644.
15. Moake JL, Byrnes JJ, Troll JH, et al. Effects of fresh-frozen plasma and its cryosupernatant fraction on von Willebrand factor multimeric forms in chronic relapsing thrombotic thrombocytopenic purpura. Blood 1985;65: 1232-6.
16. Phillips MD, Moake JL, Nolasco L, Garcia R. Plasma von Willebrand factor processing activity functions like a disultide bond reductase: reversible decrease of multimer size. Thromb Haemost 1993;69:1199. abstract.
17. Moake JL. Studies on the pathophysiology of thrombotic thrombocytopenic purpura. Semin Hematol 1997;34:83-9.
18. Dent JA, Berkowitz SD, Ware J, Kasper CK, Ruggeri ZM. Identification of a cleavage site directing the immunochemical detection of molecular abnormalities in type IIA von Willebrand factor. Proc Natl Acad Sci U S A 1990;87:6306-10. [Erratum, Proc Natl Acad Sci U S A 1990;87:9508.]
19. Bell WR, Braine HG, Ness PM, Kickler TS. Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: clinical experience of 108 patients. N Engl J Med 1991;325:398-403.
20. Moake JL, Rudy CK, Troll JH, et al. Therapy of chronic relapsing thrombotic thrombocytopenic purpura with prednisone and azathioprine. Am J Hematol 1985;20:73-9.
21. Gaddis TG, Guthrie TH Jr, Drew MJ, Sahud M, Howe RB, Mittelman A. Treatment of plasma refractory thrombotic thrombocytopenic purpura with protein A immunoabsorption. Am J Hematol 1997;55:55-8.
22. Bobbio-Pallavicini E, Porta C, Centurioni R, et al. Vincristine sulfate for the treatment of thrombotic thrombocytopenic purpura refractory to plasma-exchange. Eur J Haematol 1994;52:222-6.
23. Höffkes H-G, Weber F, Uppenkamp M, et al. Recovery by splenectomy in patients with relapsed thrombotic thrombocytopenic purpura and treatment failure to plasma exchange. Semin Thromb Hemost 1995;21:161-5.