J Neurosci Rural Pract. 2015 Jul-Sep;6(3):407-9. doi: 10.4103/0976-3147.158791.
Hypercoagulability in hereditary hemorrhagic telangiectasia with epilepsy.
Journal of neurosciences in rural practice
Josef Finsterer, Ernst Sehnal
Affiliations
Affiliations
- Department of Neurological, General Hospital Rudolfstiftung, Vienna, Austria, Europe.
- Department of Cardiology and Intensive Care Medicine, General Hospital Rudolfstiftung, Vienna, Austria, Europe.
PMID: 26167029
PMCID: PMC4481800 DOI: 10.4103/0976-3147.158791
Abstract
Recent data indicate that in patients with hereditary hemorrhagic teleangiectasia (HHT), low iron levels due to inadequate replacement after hemorrhagic iron losses are associated with elevated factor-VIII plasma levels and consecutively increased risk of venous thrombo-embolism. Here, we report a patient with HHT, low iron levels, elevated factor-VIII, and recurrent venous thrombo-embolism. A 64-year-old multimorbid Serbian gipsy was diagnosed with HHT at age 62 years. He had a history of recurrent epistaxis, teleangiectasias on the lips, renal and pulmonary arterio-venous malformations, and a family history positive for HHT. He had experienced recurrent venous thrombosis (mesenteric vein thrombosis, portal venous thrombosis, deep venous thrombosis), insufficiently treated with phenprocoumon during 16 months and gastro-intestinal bleeding. Blood tests revealed sideropenia and elevated plasma levels of coagulation factor-VIII. His history was positive for diabetes, arterial hypertension, hyperlipidemia, smoking, cerebral abscess, recurrent ischemic stroke, recurrent ileus, peripheral arterial occluding disease, polyneuropathy, mild renal insufficiency, and epilepsy. Following recent findings, hypercoagulability was attributed to the sideropenia-induced elevation of coagulation factor-VIII. In conclusion, HHT may be associated with hypercoagulability due to elevated factor-VIII associated with low serum iron levels from recurrent bleeding. Iron substitution may prevent HHT patients from hypercoagulability.
Keywords: Antithrombotic agents; blood coagulation factors; risk factors; thrombophilia; thrombosis
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