Hereditary thrombophilia / hypercoagulopathies

Heparin cofactor II deficiency

Last author update: 1 June 2012
Last staff update: 22 September 2020

Copyright: 2002-2024,, Inc.

PubMed Search: Heparin cofactor II deficiency

Jeremy C. Parsons, M.D.
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Cite this page: Parsons JC. Heparin cofactor II deficiency. website. Accessed February 26th, 2024.
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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