Hereditary thrombophilia / hypercoagulopathies
Heparin cofactor II deficiency

Topic Completed: 1 June 2012

Revised: 7 January 2019, last major update June 2012

Copyright: (c) 2002-2016,, Inc.

PubMed Search: Heparin cofactor II deficiency

Page views in 2019: 16
Page views in 2020 to date: 3
Cite this page: Parsons J. Heparin cofactor II deficiency. website. Accessed June 7th, 2020.
Definition / general
  • 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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