Hereditary thrombophilia / hypercoagulopathies
Heparin cofactor II deficiency

Author: Jeremy Parsons, M.D. (see Authors page)

Revised: 29 April 2016, last major update June 2012

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

PubMed Search: Heparin cofactor II deficiency

Cite this page: Heparin cofactor II deficiency. website. Accessed April 25th, 2018.
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