Coagulation
Hereditary thrombophilia / hypercoagulopathies
Heparin cofactor II deficiency



Topic Completed: 1 June 2012

Revised: 7 January 2019, last major update June 2012

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PubMed Search: Heparin cofactor II deficiency


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Cite this page: Parsons J. Heparin cofactor II deficiency. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/coagulationheparincofactorII.html. 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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