|
|
 Previous Article | Table of Contents | Next Article 
Blood, Vol. 93 No. 11 (June 1), 1999:
pp. 3798-3802
von Willebrand Factor Proteolysis Is Deficient in Classic, but not in
Bone Marrow Transplantation-Associated, Thrombotic Thrombocytopenic
Purpura
R. Martijn van der Plas,
Marion E. Schiphorst,
Eric G. Huizinga,
Ronald J. Hené,
Leo F. Verdonck,
Jan J. Sixma, and
Rob Fijnheer
From the Thrombosis and Haemostasis Laboratory, Department of
Haematology, Institute of Biomembranes, University Medical Centre
Utrecht, Utrecht, The Netherlands.
Thrombotic thrombocytopenic purpura (TTP) after bone marrow
transplantation (BMT) differs from classic TTP in its clinical course
and therapy. A characteristic of classic TTP is the inhibition of a
plasma protease that specifically cleaves von Willebrand factor (vWF),
thus reducing its multimeric size. We investigated whether this
protease was also inhibited in BMT-associated TTP. Plasma from patients
with classic or BMT-associated TTP was incubated with recombinant vWF
R834Q, a vWF mutant with enhanced sensitivity to the protease. The
proteolysis of vWF multimers was analyzed and quantified on Western
blot. Metalloprotease activity was strongly inhibited in the classic
TTP patient group. However, metalloprotease activity was normal in the
BMT-associated TTP patient group. The difference in activity between
the two patient groups was highly significant (P = .0016).
The results indicate that the etiologies of classic and BMT-associated
TTP are indeed different and provide an explanation for the lack of
success of plasma exchange in BMT-associated TTP.
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
J. E. Sadler
Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura
Blood,
July 1, 2008;
112(1):
11 - 18.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. K. Chauhan, J. Kisucka, M. R. Cozzi, M. T. Walsh, F. A. Moretti, M. Battiston, M. Mazzucato, L. De Marco, F. E. Baralle, D. D. Wagner, et al.
Prothrombotic Effects of Fibronectin Isoforms Containing the EDA Domain
Arterioscler. Thromb. Vasc. Biol.,
February 1, 2008;
28(2):
296 - 301.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
L. Heemskerk, N. Scott-Douglas, S. Yilmaz, and K. McLaughlin
Resolution of thrombotic microangiopathy following renal transplant
Nephrol. Dial. Transplant.,
March 1, 2005;
20(3):
639 - 641.
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. K. Vesely, J. N. George, B. Lammle, J.-D. Studt, L. Alberio, M. A. El-Harake, and G. E. Raskob
ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients
Blood,
July 1, 2003;
102(1):
60 - 68.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
H.-M. Tsai
Advances in the Pathogenesis, Diagnosis, and Treatment of Thrombotic Thrombocytopenic Purpura
J. Am. Soc. Nephrol.,
April 1, 2003;
14(4):
1072 - 1081.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
H.-M. Tsai
Platelet Activation and the Formation of the Platelet Plug: Deficiency of ADAMTS13 Causes Thrombotic Thrombocytopenic Purpura
Arterioscler. Thromb. Vasc. Biol.,
March 1, 2003;
23(3):
388 - 396.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. J. Bodnar, X. Xi, Z. Li, M. C. Berndt, and X. Du
Regulation of Glycoprotein Ib-IX-von Willebrand Factor Interaction by cAMP-dependent Protein Kinase-mediated Phosphorylation at Ser 166 of Glycoprotein Ibbeta
J. Biol. Chem.,
November 27, 2002;
277(49):
47080 - 47087.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
T. G. DeLoughery
Thrombocytopenia in Critical Care Patients
J Intensive Care Med,
November 1, 2002;
17(6):
267 - 282.
[Abstract]
[PDF]
|
|
|
|
|
|
|
J. L. Moake
Thrombotic Microangiopathies
N. Engl. J. Med.,
August 22, 2002;
347(8):
589 - 600.
[Full Text]
[PDF]
|
|
|
|
|
|
|
T. J. Raife, S. R. Lentz, B. S. Atkinson, S. K. Vesely, and M. J. Hessner
Factor V Leiden: a genetic risk factor for thrombotic microangiopathy in patients with normal von Willebrand factor-cleaving protease activity
Blood,
January 15, 2002;
99(2):
437 - 442.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. N. George, J. E. Sadler, and B. Lammle
Platelets: Thrombotic Thrombocytopenic Purpura
Hematology,
January 1, 2002;
2002(1):
315 - 334.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
P. M. Mannucci, M. T. Canciani, I. Forza, F. Lussana, A. Lattuada, and E. Rossi
Changes in health and disease of the metalloprotease that cleaves von Willebrand factor
Blood,
November 1, 2001;
98(9):
2730 - 2735.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. Fujikawa, H. Suzuki, B. McMullen, and D. Chung
Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family
Blood,
September 15, 2001;
98(6):
1662 - 1666.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Veyradier, B. Obert, A. Houllier, D. Meyer, and J.-P. Girma
Specific von Willebrand factor-cleaving protease in thrombotic microangiopathies: a study of 111 cases
Blood,
September 15, 2001;
98(6):
1765 - 1772.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. D.J.M. Kanters, J.-D. Banga, A. Algra, R. C.J.M. Frijns, J. J. Beutler, and R. Fijnheer
Plasma Levels of Cellular Fibronectin in Diabetes
Diabetes Care,
February 1, 2001;
24(2):
323 - 327.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
K. R. McCrae, J. B. Bussel, P. M. Mannucci, G. Remuzzi, and D. B. Cines
Platelets: An Update on Diagnosis and Management of Thrombocytopenic Disorders
Hematology,
January 1, 2001;
2001(1):
282 - 305.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S L Allford and S J Machin
Current understanding of the pathophysiology of thrombotic thrombocytopenic purpura
J. Clin. Pathol.,
July 1, 2000;
53(7):
497 - 501.
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Furlan;, R. Fijnheer, M. van der Plas, E. Huizinga, R. Hene, J. Sixma, and L. Verdonck
von Willebrand Factor Proteolysis in Thrombotic Thrombocytopenic Purpura
Blood,
November 15, 1999;
94(10):
3611 - 3613.
[Full Text]
[PDF]
|
|
|
| |
