|
|
Blood, 15 January 2009, Vol. 113, No. 3, pp. 526-534.
Prepublished online as a Blood First Edition Paper on September 19, 2008; DOI 10.1182/blood-2008-04-152280.
 Previous Article | Table of Contents | Next Article 
CLINICAL TRIALS AND OBSERVATIONS
Clinical and molecular predictors of thrombocytopenia and risk of bleeding in patients with von Willebrand disease type 2B: a cohort study of 67 patients
Augusto B. Federici1,
Pier M. Mannucci1,
Giancarlo Castaman2,
Luciano Baronciani1,
Paolo Bucciarelli1,
Maria T. Canciani1,
Alessandro Pecci3,
Peter J. Lenting4, and
Philip G. De Groot4
1 Angelo Bianchi Bonomi Hemophilia Thrombosis Center, Department of Medicine and Medical Specialties, Istituti di Ricovero e Cura a Carattere Scientifico (IRCCS) Maggiore Hospital, Mangiagalli and Regina Elena Foundation, and University of Milan, Milan, Italy;
2 Department of Hematology, Hemophilia Thrombosis Center, San Bortolo Hospital, Vicenza, Italy;
3 Department of Internal Medicine, IRCCS Policlinico S Matteo Foundation and University of Pavia, Pavia, Italy; and
4 Department of Clinical Chemistry and Hematology, University of Utrecht, Utrecht, The Netherlands
Type 2B von Willebrand disease (VWD2B) is caused by an abnormal von Willebrand factor (VWF) with increased affinity for the platelet receptor glycoprotein Ib- (GPIb-) that may result in moderate to severe thrombocytopenia. We evaluated the prevalence and clinical and molecular predictors of thrombocytopenia in a cohort of 67 VWD2B patients from 38 unrelated families characterized by VWF mutations. Platelet count, mean platelet volume, and morphologic evaluations of blood smear were obtained at baseline and during physiologic (pregnancy) or pathologic (infections, surgeries) stress conditions. Thrombocytopenia was found in 20 patients (30%) at baseline and in 38 (57%) after stress conditions, whereas platelet counts were always normal in 16 patients (24%) from 5 families carrying the P1266L/Q or R1308L mutations. VWF in its GPIb-–binding conformation (VWF–GPIb-/BC) was higher than normal in all except the 16 cases without thrombocytopenia (values up to 6-fold higher than controls). The risk of bleeding was higher in patients with thrombocytopenia (adjusted hazard ratio = 4.57; 95% confidence interval, 1.17-17.90) and in those with the highest tertile of bleeding severity score (5.66; 95% confidence interval, 1.03-31.07). Prediction of possible thrombocytopenia in VWD2B by measuring VWF–GPIb-/BC is important because a low platelet count is an independent risk factor for bleeding.
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
Related Article in Blood Online:
-
At least Type VWD2B is a discrete variant of VWD, isn't it?
- Robert R. Montgomery
Blood 2009 113: 501.
[Full Text]
[PDF]
This article has been cited by other articles:
|
|
|
|
 
P. Nurden, A. T Nurden, S. La Marca, M. Punzo, L. Baronciani, and A. B. Federici
Platelet morphological changes in 2 patients with von Willebrand disease type 3 caused by large homozygous deletions of the von Willebrand factor gene
Haematologica,
November 1, 2009;
94(11):
1627 - 1629.
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. F. De Meyer, H. Deckmyn, and K. Vanhoorelbeke
von Willebrand factor to the rescue
Blood,
May 21, 2009;
113(21):
5049 - 5057.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. B Federici and M. T. Canciani
Clinical and laboratory versus molecular markers for a correct classification of von Willebrand disease
Haematologica,
May 1, 2009;
94(5):
610 - 615.
[Full Text]
[PDF]
|
|
|
|
|
|
|
P. Nurden
The VWD2B saga continues to Montreal
Blood,
April 2, 2009;
113(14):
3134 - 3135.
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. C. Jackson, G. D. Sinclair, S. Cloutier, Z. Duan, M. L. Rand, and M.-C. Poon
The Montreal platelet syndrome kindred has type 2B von Willebrand disease with the VWF V1316M mutation
Blood,
April 2, 2009;
113(14):
3348 - 3351.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
