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[Clotting factor VIII in Sneddon syndrome]
Klinicheskaia Meditsina
|November 6, 2003
Summary
Hyperactivity of coagulation factor VIII (fVIII) is common in Sneddon's syndrome (SS), increasing thrombosis risk. This elevated fVIII activity, independent of antiphospholipid antibodies, drives thrombotic events in SS patients.
Area of Science:
- Hematology
- Vascular Medicine
- Rheumatology
Context:
- Sneddon's syndrome (SS) presents with clinical features mimicking primary antiphospholipid syndrome (PAS), including ischemic cerebrovascular disorders and livedo reticularis.
- Coagulation factor VIII (fVIII) hyperactivity is a known contributor to hypercoagulable states.
Purpose:
- To investigate the role of coagulation factor VIII (fVIII) activity in the pathogenesis of thrombosis in patients with Sneddon's syndrome (SS).
Summary:
- Hyperactivity of coagulation factor VIII (fVIII) was observed in 75% of 28 Sneddon's syndrome patients.
- Elevated fVIII activity correlated with increased incidence of ischemic strokes, recurrent strokes, vascular dementia, and peripheral venous thromboses.
- High fVIII activity was noted even in antiphospholipid-antibody-negative patients, suggesting an intrinsic mechanism for thrombosis in SS.
Impact:
- Demonstrates that elevated fVIII activity is a key mechanism driving thrombosis in Sneddon's syndrome.
- Suggests a potential interaction between fVIII and specific antiphospholipid antibodies, rendering fVIII resistant to protein C inactivation.
- Highlights the importance of assessing fVIII activity in SS patients for risk stratification and management of thrombotic events.