|
|
 Previous Article | Table of Contents | Next Article 
Blood, Vol. 94 No. 3 (August 1), 1999:
pp. 895-901
Extensive Venous and Arterial Thrombosis Associated With an
Inhibitor to Activated Protein C
Ariella Zivelin,
Sanford Gitel,
John H. Griffin,
Xiao Xu,
Jose A. Fernandez,
Uri Martinowitz,
Yael Cohen,
Hillel Halkin,
Uri Seligsohn, and
Aida Inbal
From the Institute of Thrombosis and Hemostasis, Departments of
Hematology and Internal Medicine, Sheba Medical Center, Tel-Hashomer,
Israel; and the Department of Molecular and Experimental Medicine, The
Scripps Research Institute, La Jolla, CA.
Activated protein C resistance (APCR) in the absence of alterations
in the factor V gene has been observed during pregnancy, in patients on
oral contraceptives, in the presence of antiphospholipid antibodies,
and in patients with ischemic stroke. We report a 49-year-old woman
with recurrent major venous and arterial thromboses who displayed
pronounced APCR, yet no changes in the activated protein C (APC)
cleavage sites of factor V. The APCR values determined by four
different assays were similar to those obtained in plasma from a
homozygote for factor V Q506. Addition of IgG isolated from the
patient's serum to normal plasma lowered the APCR ratio from 2.4 to
1.6. Incubation of patient's IgG with normal APC resulted in a
profound change in the mobility of APC in crossed
immunoelectrophoresis. APC was also shown to bind to patient's IgG
immobilized on a protein A agarose column. Factor Va inactivation by
APC was inhibited by patient's IgG, but not by control IgG in the
presence or absence of either phospholipids or protein S. These results
provide evidence for the existence of an acquired antibody against APC
in the patient's plasma, which gave rise to the APCR phenotype and was
probably responsible for the major thrombotic events. We suggest that
acquired APCR due to anti-APC antibodies be considered a potential
cause for severe venous and arterial thromboses.
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
M-H Tsai, W-P Tsai, S-T Liao, and L-b Liou
Anticardiolipin antibodies in various diseases in Taiwan: a retrospective analysis
Lupus,
October 1, 2003;
12(10):
747 - 753.
[Abstract]
[PDF]
|
|
|
|
|
|
|
H. Deguchi, J. A. Fernandez, and J. H. Griffin
Neutral Glycosphingolipid-dependent Inactivation of Coagulation Factor Va by Activated Protein C and Protein S
J. Biol. Chem.,
March 8, 2002;
277(11):
8861 - 8865.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
U. Seligsohn and A. Lubetsky
Genetic Susceptibility to Venous Thrombosis
N. Engl. J. Med.,
April 19, 2001;
344(16):
1222 - 1231.
[Full Text]
[PDF]
|
|
|
|
|
|
|
H. Deguchi, J. A. Fernandez, I. Pabinger, J. A. Heit, and J. H. Griffin
Plasma glucosylceramide deficiency as potential risk factor for venous thrombosis and modulator of anticoagulant protein C pathway
Blood,
April 1, 2001;
97(7):
1907 - 1914.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. Male, L. Mitchell, J. Julian, P. Vegh, P. Joshua, M. Adams, M. David, and M. E. Andrew
Acquired activated protein C resistance is associated with lupus anticoagulants and thrombotic events in pediatric patients with systemic lupus erythematosus
Blood,
February 15, 2001;
97(4):
844 - 849.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. Kovacs, T. L Lafferty, L. H Brent, and R. J DeHoratius
Transverse myelopathy in systemic lupus erythematosus: an analysis of 14 cases and review of the literature
Ann Rheum Dis,
February 1, 2000;
59(2):
120 - 124.
[Abstract]
[Full Text]
|
|
|
|
|
