Platelet reactive conformation and multimeric pattern of von Willebrand factor in acquired thrombotic thrombocytopenic purpura during acute disease and remission

Journal of Thrombosis and Haemostasis

Volumn 9, Issue 9, 2011, Pages 1744-1751

  Lotta, L A ^a   Lombardi, R ^a   Mariani, M ^a   Lancellotti, S ^b   De Cristofaro, R ^b   Hollestelle, M J ^c   Canciani, M T ^a   Mannucci, P M ^a   Peyvandi, F ^a  

^a FONDAZIONE IRCCS CA GRANDA OSPEDALE MAGGIORE POLICLINICO   (Italy)

^b CATHOLIC UNIVERSITY   (Italy)

^c UNIVERSITY MEDICAL CENTER UTRECHT   (Netherlands)

Author keywords

ADAMTS 13; Multimers; Platelet reactive von Willebrand factor; Thrombotic thrombocytopenic purpura; Von Willebrand factor

Indexed keywords

COLLAGEN; GLYCOPROTEIN IB ALPHA; HEMOGLOBIN; LACTATE DEHYDROGENASE; RISTOCETIN; VON WILLEBRAND FACTOR; VON WILLEBRAND FACTOR CLEAVING PROTEINASE;

ARTICLE; BLOOD GROUP O; CONTROLLED STUDY; DISEASE ACTIVITY; DISEASE SEVERITY; HUMAN; LACTATE DEHYDROGENASE BLOOD LEVEL; MAJOR CLINICAL STUDY; PRIORITY JOURNAL; PROTEIN BINDING; PROTEIN BLOOD LEVEL; REMISSION; RETROSPECTIVE STUDY; THROMBOCYTE; THROMBOCYTE COUNT; THROMBOTIC THROMBOCYTOPENIC PURPURA;

ACUTE DISEASE; ADAM PROTEINS; BLOOD PLATELETS; CASE-CONTROL STUDIES; COLLAGEN; HUMANS; MEMBRANE GLYCOPROTEINS; PROTEIN BINDING; PROTEIN CONFORMATION; PROTEIN MULTIMERIZATION; PURPURA, THROMBOTIC THROMBOCYTOPENIC; RETROSPECTIVE STUDIES; VON WILLEBRAND FACTOR;

EID: 80052396255     PISSN: 15387933     EISSN: 15387836     Source Type: Journal    
DOI: 10.1111/j.1538-7836.2011.04428.x     Document Type: Article

References (19)

1. Sadler JE. von Willebrand factor, ADAMTS-13, and thrombotic thrombocytopenic purpura. Blood 2008; 112: 11-8.
2. Dong JF, Moake JL, Nolasco L, Bernardo A, Arceneaux W, Shrimpton CN, Schade AJ, McIntire LV, Fujikawa K, López JA. ADAMTS-13 rapidly cleaves newly secreted ultralarge von Willebrand factor multimers on the endothelial surface under flowing conditions. Blood 2002; 100: 4033-9.
3. Moake JL. Thrombotic microangiopathies. N Engl J Med 2002; 347: 589-600.
4. Lotta LA, Garagiola I, Palla R, Cairo A, Peyvandi F. ADAMTS-13 mutations and polymorphisms in congenital thrombotic thrombocytopenic purpura. Hum Mutat 2010; 31: 11-9.
5. Hovinga JA, Vesely SK, Terrell DR, Lammle B, George JN. Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood 2010; 115: 1500-11.
6. Motto DG, Chauhan AK, Zhu G, Homeister J, Lamb CB, Desch KC, Zhang W, Tsai HM, Wagner DD, Ginsburg D. Shigatoxin triggers thrombotic thrombocytopenic purpura in genetically susceptible ADAMTS-13-deficient mice. J Clin Invest 2005; 115: 2752-61.
7. Hulstein JJ, de Groot PG, Silence K, Veyradier A, Fijnheer R, Lenting PJ. A novel nanobody that detects the gain-of-function phenotype of von Willebrand factor in ADAMTS-13 deficiency and von Willebrand disease type 2B. Blood 2005; 106: 3035-42.
8. Groot E, Fijnheer R, Sebastian SA, de Groot PG, Lenting PJ. The active conformation of von Willebrand factor in patients with thrombotic thrombocytopenic purpura in remission. J Thromb Haemost 2009; 7: 962-9.
9. Lotta LA, Mariani M, Consonni D, Mancini I, Palla R, Maino A, Vucelic D, Pizzuti M, Mannucci PM, Peyvandi F. Different clinical severity of first episodes and recurrences of thrombotic thrombocytopenic purpura. Br J Haematol 2010; 151: 488-94.
10. Peyvandi F, Lavoretano S, Palla R, Valsecchi C, Merati G, De Cristofaro R, Rossi E, Mannuccio Mannucci P. Mechanisms of the interaction between two ADAMTS-13 gene mutations leading to severe deficiency of enzymatic activity. Hum Mutat 2006; 27: 330-6.
11. Budde U, Schneppenheim R. von Willebrand factor and von Willebrand disease. Rev Clin Exp Hematol 2001; 5: 335-68.
12. Federici AB, Mannucci PM, Castaman G, Baronciani L, Bucciarelli P, Canciani MT, Pecci A, Lenting PJ, De Groot PG. Clinical and molecular predictors of thrombocytopenia and risk of bleeding in patients with von Willebrand disease type 2B: a cohort study of 67 patients. Blood 2009; 113: 526-34.
13. Peyvandi F, Lavoretano S, Palla R, Feys HB, Vanhoorelbeke K, Battaglioli T, Valsecchi C, Canciani MT, Fabris F, Zver S, Réti M, Mikovic D, Karimi M, Giuffrida G, Laurenti L, Mannucci PM. ADAMTS-13 and anti-ADAMTS-13 antibodies as markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission. Haematologica 2008; 93: 232-9.
14. Baronciani L, Federici AB, Cozzi G, Canciani MT, Mannucci PM. von Willebrand factor collagen binding assay in von Willebrand disease type 2A, 2B, and 2M. J Thromb Haemost 2006; 4: 2088-90.
15. Moake JL, Rudy CK, Troll JH, Weinstein MJ, Colannino NM, Azocar J, Seder RH, Hong SL, Deykin D. Unusually large plasma factor VIII:von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura. N Engl J Med 1982; 307: 1432-5.
16. Rowe JM, Francis CW, Cyran EM, Marder VJ. Thrombotic thrombocytopenic purpura: recovery after splenectomy associated with persistence of abnormally large von Willebrand factor multimers. Am J Hematol 1985; 20: 161-8.
17. Murphy WG, Moore JC, Barr RD, Pai MK, Kelton JG. Relationship between platelet aggregating factor and von Willebrand factor in thrombotic thrombocytopenic purpura. Br J Haematol 1987; 66: 509-13.
18. Moake JL, Rudy CK, Troll JH, Weinstein MJ, Colannino NM, Hong SL, Koutcher JA, Melaragno AJ, Manner CE. von Willebrand factor abnormalities and endothelial cell perturbation in a patient with acute thrombotic thrombocytopenic purpura. Am J Med Sci 1986; 291: 47-50.
19. Moake JL, McPherson PD. Abnormalities of von Willebrand factor multimers in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. Am J Med 1989; 87: 9N-15N.