Coagulation
Hereditary thrombophilia / hypercoagulopathies
Heparin cofactor II deficiency
Author: Jeremy C. Parsons, M.D.
Last author update: 1 June 2012
Last staff update: 22 September 2020
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: Heparin cofactor II deficiency
Cite this page: Parsons JC. Heparin cofactor II deficiency. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/coagulationheparincofactorII.html. Accessed April 19th, 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
- Family with homozygous deficiency, but no significant symptoms (Circulation 2004;110:1303)
