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Published on: September 9, 2012
Heparin cofactor II deficiency
1Hematology Division, Department of Medicine, Washington University School of Medicine, St Louis, Mo 63110, USA. tollefsen@im.wustl.edu
Insights
Congenital heparin cofactor II deficiency is not recommended for testing in patients with thromboembolic disease due to insufficient evidence. Further research is needed to establish its role as a thrombotic risk factor.
Area of Science:
- Hematology
- Thrombosis Research
- Diagnostic Medicine
Background:
- Congenital heparin cofactor II deficiency is a rare inherited disorder.
- Its role as a risk factor for thrombosis requires further investigation.
- Current diagnostic approaches for thrombophilia need evaluation.
Purpose of the Study:
- To review the current literature on heparin cofactor II deficiency and thrombosis.
- To establish expert consensus on the diagnostic utility of heparin cofactor II deficiency testing.
- To provide recommendations for laboratory assay use in assessing thrombotic risk.
Main Methods:
- Comprehensive review of the medical literature from the past decade.
- Expert consensus development at the College of American Pathologists Conference XXXVI.
- Critical assessment of clinical study design and laboratory methodologies.
Main Results:
- The literature review did not provide sufficient evidence to link heparin cofactor II deficiency to increased thrombotic risk.
- Expert consensus confirmed the lack of data supporting routine testing for this deficiency.
- Current laboratory assays are not recommended for this specific diagnostic purpose.
Conclusions:
- There is insufficient evidence to recommend testing for congenital heparin cofactor II deficiency in patients presenting with thromboembolic disease.
- The clinical significance of heparin cofactor II deficiency as a thrombotic risk factor remains unproven.
- Further research is warranted to clarify the association between heparin cofactor II deficiency and thrombosis.
Objectives:
To review of the state of the art relating to congenital heparin cofactor II deficiency as a potential risk factor for thrombosis, as reflected by the medical literature and the consensus opinion of recognized experts in the field, and to make recommendations for the use of laboratory assays for assessing this thrombotic risk in individual patients.
Data Sources:
Review of the medical literature, primarily from the last 10 years.
Data Extraction And Synthesis:
After an initial assessment of the literature, including review of clinical study design and laboratory methods, a draft manuscript was prepared and circulated to participants in the College of American Pathologists Conference XXXVI: Diagnostic Issues in Thrombophilia. Recommendations were accepted if a consensus of experts attending the conference was reached. The results of the discussion were used to revise the manuscript into its final form.
Conclusions:
Consensus was reached that there is insufficient evidence to recommend testing for heparin cofactor II deficiency in patients with thromboembolic disease.
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