Von Willebrand factor activation, granzyme-B and thrombocytopenia in meningococcal disease

Journal of Thrombosis and Haemostasis

Volumn 8, Issue 5, 2010, Pages 1098-1106

  Hollestelle, M J ^a   Sprong, T ^b   BovenschenN , ^a   de mast, Q ^b   van der ven, A J ^b   Joosten, L A B ^b   Neeleman, C ^b   Hyseni, A ^a   Lenting, P J ^c   de groot, P G ^a   van deuren, M ^b  

^a UNIVERSITY MEDICAL CENTER UTRECHT   (Netherlands)

^b RADBOUD UNIVERSITY MEDICAL CENTER   (Netherlands)

^c INSERM   (France)

Author keywords

Granzyme B; Meningococcal disease; Sepsis; Thrombocytopenia; VWF

Indexed keywords

GRANZYME B; VON WILLEBRAND FACTOR; VON WILLEBRAND FACTOR CLEAVING PROTEINASE; ADAM PROTEIN; ADAMTS13 PROTEIN, HUMAN; GRANZYME;

ARTICLE; CHILD; CLINICAL ARTICLE; CONTROLLED STUDY; FEMALE; HOSPITAL ADMISSION; HUMAN; MALE; MENINGOCOCCOSIS; PATHOPHYSIOLOGY; PRESCHOOL CHILD; PRIORITY JOURNAL; PROTEIN CLEAVAGE; SHOCK; THROMBOCYTOPENIA; BACTERIAL MENINGITIS; COMPLICATION; ENZYMOLOGY; INFANT; METABOLISM;

ADAM PROTEINS; CHILD; CHILD, PRESCHOOL; FEMALE; GRANZYMES; HUMANS; INFANT; MALE; MENINGITIS, BACTERIAL; THROMBOCYTOPENIA; VON WILLEBRAND FACTOR;

EID: 77953799912     PISSN: 15387933     EISSN: 15387836     Source Type: Journal    
DOI: 10.1111/j.1538-7836.2010.03811.x     Document Type: Article

References (32)

1. van Deuren M, Brandtzaeg P, van der Meer JW. Update on meningococcal disease with emphasis on pathogenesis and clinical management. Clin Microbiol Rev 2000, 13:144-66.
2. Nadel S, Goldstein B, Williams MD, Dalton H, Peters M, Macias WL, Abd-Allah SA, Levy H, Angle R, Wang D, Sundin DP, Giroir B. Drotrecogin alfa (activated) in children with severe sepsis: a multicentre phase III randomised controlled trial. Lancet 2007, 369:836-43.
3. Zenz W, Zoehrer B, Levin M, Fanconi S, Hatzis TD, Knight G, Mullner M, Faust SN. Use of recombinant tissue plasminogen activator in children with meningococcal purpura fulminans: a retrospective study. Crit Care Med 2004, 32:1777-80.
4. van Deuren M, Neeleman C, van't Hek LG, van der Meer JW. A normal platelet count at admission in acute meningococcal disease does not exclude a fulminant course. Intensive Care Med 1998, 24:157-61.
5. Ward PA. The dark side of C5a in sepsis. Nat Rev Immunol 2004, 4:133-42.
6. Nieuwland R, Berckmans RJ, McGregor S, Boing AN, Romijn FP, Westendorp RG, Hack CE, Sturk A. Cellular origin and procoagulant properties of microparticles in meningococcal sepsis. Blood 2000, 95:930-5.
7. Kremer Hovinga JA, Zeerleder S, Kessler P, Romani de WT, van Mourik JA, Hack CE, ten Cate H, Reitsma PH, Wuillemin WA, Lammle B. ADAMTS-13, von Willebrand factor and related parameters in severe sepsis and septic shock. J Thromb Haemost 2007, 5:2284-90.
8. Claus RA, Bockmeyer CL, Budde U, Kentouche K, Sossdorf M, Hilberg T, Schneppenheim R, Reinhart K, Bauer M, Brunkhorst FM, Losche W. Variations in the ratio between von Willebrand factor and its cleaving protease during systemic inflammation and association with severity and prognosis of organ failure. Thromb Haemost 2009, 101:239-47.
9. Bockmeyer CL, Claus RA, Budde U, Kentouche K, Schneppenheim R, Losche W, Reinhart K, Brunkhorst FM. Inflammation-associated ADAMTS13 deficiency promotes formation of ultra-large von Willebrand factor. Haematologica 2008, 93:137-40.
10. Martin K, Borgel D, Lerolle N, Feys HB, Trinquart L, Vanhoorelbeke K, Deckmyn H, Legendre P, Diehl JL, Baruch D. Decreased ADAMTS-13 (A disintegrin-like and metalloprotease with thrombospondin type 1 repeats) is associated with a poor prognosis in sepsis-induced organ failure. Crit Care Med 2007, 35:2375-82.
11. Hazelzet JA, Risseeuw-Appel IM, Kornelisse RF, Hop WC, Dekker I, Joosten KF, de GR, Hack CE. Age-related differences in outcome and severity of DIC in children with septic shock and purpura. Thromb Haemost 1996, 76:932-8.
12. Ruggeri ZM. Structure and function of von Willebrand factor. Thromb Haemost 1999, 82:576-84.
13. Wagner DD, Fay PJ, Sporn LA, Sinha S, Lawrence SO, Marder VJ. Divergent fates of von Willebrand factor and its propolypeptide (von Willebrand antigen II) after secretion from endothelial cells. Proc Natl Acad Sci U S A 1987, 84:1955-9.
14. Groot E, de Groot PG, Fijnheer R, Lenting PJ. The presence of active von Willebrand factor under various pathological conditions. Curr Opin Hematol 2007, 14:284-9.
15. van Mourik JA, Boertjes R, Huisveld IA, Fijnvandraat K, Pajkrt D, van Genderen PJ, Fijnheer R. von Willebrand factor propeptide in vascular disorders: a tool to distinguish between acute and chronic endothelial cell perturbation. Blood 1999, 94:179-85.
16. Ono T, Mimuro J, Madoiwa S, Soejima K, Kashiwakura Y, Ishiwata A, Takano K, Ohmori T, Sakata Y. Severe secondary deficiency of von Willebrand factor-cleaving protease (ADAMTS13) in patients with sepsis-induced disseminated intravascular coagulation: its correlation with development of renal failure. Blood 2006, 107:528-34.
17. Nguyen TC, Liu A, Liu L, Ball C, Choi H, May WS, Aboulfatova K, Bergeron AL, Dong JF. Acquired ADAMTS-13 deficiency in pediatric patients with severe sepsis. Haematologica 2007, 92:121-4.
18. Buzza MS, Dyson JM, Choi H, Gardiner EE, Andrews RK, Kaiserman D, Mitchell CA, Berndt MC, Dong JF, Bird PI. Antihemostatic activity of human granzyme B mediated by cleavage of von Willebrand factor. J Biol Chem 2008, 283:22498-504.
19. Choy JC, McDonald PC, Suarez AC, Hung VH, Wilson JE, McManus BM, Granville DJ. Granzyme B in atherosclerosis and transplant vascular disease: association with cell death and atherosclerotic disease severity. Mod Pathol 2003, 16:460-70.
20. Goldstein B, Giroir B, Randolph A. International pediatric sepsis consensus conference: definitions for sepsis and organ dysfunction in pediatrics. Pediatr Crit Care Med 2005, 6:2-8.
21. van Brakel MJ, van Vught AJ, Gemke RJ. Pediatric risk of mortality (PRISM) score in meningococcal disease. Eur J Pediatr 2000, 159:232-6.
22. Taylor FB, Toh CH, Hoots WK, Wada H, Levi M. Towards definition, clinical and laboratory criteria, and a scoring system for disseminated intravascular coagulation. Thromb Haemost 2001, 86:1327-30.
23. 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 ADAMTS13 deficiency and von Willebrand disease type 2B. Blood 2005, 106:3035-42.
24. van Schooten CJ, Denis CV, Lisman T, Eikenboom JC, Leebeek FW, Goudemand J, Fressinaud E, van den Berg HM, de Groot PG, Lenting PJ. Variations in glycosylation of von Willebrand factor with O-linked sialylated T antigen are associated with its plasma levels. Blood 2007, 109:2430-7.
25. Bovenschen N, de Koning PJ, Quadir R, Broekhuizen R, Damen JM, Froelich CJ, Slijper M, Kummer JA. NK cell protease granzyme M targets alpha-tubulin and disorganizes the microtubule network. J Immunol 2008, 180:8184-91.
26. Rubin DB, Wiener-Kronish JP, Murray JF, Green DR, Turner J, Luce JM, Montgomery AB, Marks JD, Matthay MA. Elevated von Willebrand factor antigen is an early plasma predictor of acute lung injury in nonpulmonary sepsis syndrome. J Clin Invest 1990, 86:474-80.
27. Kayal S, Jais JP, Aguini N, Chaudiere J, Labrousse J. Elevated circulating E-selectin, intercellular adhesion molecule 1, and von Willebrand factor in patients with severe infection. Am J Respir Crit Care Med 1998, 157:776-84.
28. Jilma B, Blann A, Pernerstorfer T, Stohlawetz P, Eichler HG, Vondrovec B, Amiral J, Richter V, Wagner OF. Regulation of adhesion molecules during human endotoxemia. No acute effects of aspirin. Am J Respir Crit Care Med 1999, 159:857-63.
29. Nossent AY, van Marion V, van Tilburg NH, Rosendaal FR, Bertina RM, van Mourik JA, Eikenboom HC. von Willebrand factor and its propeptide: the influence of secretion and clearance on protein levels and the risk of venous thrombosis. J Thromb Haemost 2006, 4:2556-62.
30. van Woensel JB, Biezeveld MH, Hack CE, Bos AP, Kuijpers TW. Elastase and granzymes during meningococcal disease in children: correlation to disease severity. Intensive Care Med 2005, 31:1239-47.
31. Freishtat RJ, Natale J, Benton AS, Cohen J, Sharron M, Wiles AA, Ngor WM, Mojgani B, Bradbury M, Degnan A, Sachdeva R, Debiase LM, Ghimbovschi S, Chow M, Bunag C, Kristosturyan E, Hoffman EP. Sepsis alters the megakaryocyte-platelet transcriptional axis resulting in granzyme B-mediated lymphotoxicity. Am J Respir Crit Care Med 2009, 179:467-73.
32. Arya M, Anvari B, Romo GM, Cruz MA, Dong JF, McIntire LV, Moake JL, Lopez JA. Ultralarge multimers of von Willebrand factor form spontaneous high-strength bonds with the platelet glycoprotein Ib-IX complex: studies using optical tweezers. Blood 2002, 99:3971-7.