Cite this page: Parsons JC. Heparin cofactor II deficiency. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/coagulationheparincofactorII.html. Accessed October 18th, 2021.
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)
- Family with homozygous deficiency, but no significant symptoms (Circulation 2004;110:1303)