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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.
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