Coagulation - Heparin cofactor II deficiency

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Coagulation

Hereditary thrombophilia / hypercoagulopathies

Heparin cofactor II deficiency

Reviewer: Jeremy Parsons, M.D. (see Reviewers page)
Revised: 13 June 2012, last major update June 2012
Copyright: (c) 2002-2012, PathologyOutlines.com, Inc.

General
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● Very rare hypercoagulable condition, either hereditary (autosomal dominant, 15 families documented through 2002) or acquired (liver disease)
● Associated with thrombosis, but not a strong risk factor by itself
● Testing patients with thromboembolic disease for heparin cofactor II deficiency is not recommended as a first or second line test (Arch Pathol Lab Med 2002;126:1394)

Case reports
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● Family with homozygous deficiency, but no significant symptoms (Circulation 2004;110:1303)

End of Coagulation > Hereditary thrombophilia / hypercoagulopathies > Heparin cofactor II deficiency

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