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Hageman factor deficiency presentation and implications for management.
1St. Vincent's Medical Center, Bridgeport.
Connecticut Medicine
|September 1, 1992
Summary
Factor XII deficiency, an inherited coagulation disorder, presents diagnostic challenges. Management requires specific monitoring methods, especially during heparin therapy, to ensure patient safety.
Area of Science:
- Hematology
- Coagulation Disorders
Background:
- Factor XII deficiency is an inherited autosomal recessive coagulation disorder.
- It presents diagnostic challenges in patient management.
- Clinical suspicion arises from prolonged activated partial thromboplastin time (APTT) with normal prothrombin time (PT) and bleeding time.
Purpose of the Study:
- To discuss the clinical significance and management of Factor XII deficiency.
- To highlight diagnostic confirmation methods.
- To review therapeutic monitoring strategies, particularly during heparinization.
Main Methods:
- Clinical suspicion based on coagulation profile (prolonged APTT, normal PT, normal bleeding time).
- Diagnostic confirmation via normalization of PTT with aged plasma and factor assay.
- Monitoring heparin therapy using chromogenic heparin assay, citrated thrombin time, and recalcified thrombin time.
Main Results:
- Factor XII deficiency can be suspected clinically and confirmed through specific laboratory tests.
- Effective monitoring strategies exist for heparin therapy in patients with this deficiency.
- Two illustrative cases are presented to demonstrate clinical scenarios.
Conclusions:
- Understanding Factor XII deficiency is crucial for accurate diagnosis and effective patient management.
- Specific laboratory tests are essential for confirmation.
- Appropriate monitoring is vital when managing patients with Factor XII deficiency, especially during anticoagulation therapy.