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Hypercoagulability in hereditary hemorrhagic telangiectasia with epilepsy

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 p...

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Autores principales: Finsterer, Josef, Sehnal, Ernst
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4481800/
https://www.ncbi.nlm.nih.gov/pubmed/26167029
http://dx.doi.org/10.4103/0976-3147.158791
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author Finsterer, Josef
Sehnal, Ernst
author_facet Finsterer, Josef
Sehnal, Ernst
author_sort Finsterer, Josef
collection PubMed
description 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.
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spelling pubmed-44818002015-07-12 Hypercoagulability in hereditary hemorrhagic telangiectasia with epilepsy Finsterer, Josef Sehnal, Ernst J Neurosci Rural Pract Case Report 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. Medknow Publications & Media Pvt Ltd 2015 /pmc/articles/PMC4481800/ /pubmed/26167029 http://dx.doi.org/10.4103/0976-3147.158791 Text en Copyright: © Journal of Neurosciences in Rural Practice http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Finsterer, Josef
Sehnal, Ernst
Hypercoagulability in hereditary hemorrhagic telangiectasia with epilepsy
title Hypercoagulability in hereditary hemorrhagic telangiectasia with epilepsy
title_full Hypercoagulability in hereditary hemorrhagic telangiectasia with epilepsy
title_fullStr Hypercoagulability in hereditary hemorrhagic telangiectasia with epilepsy
title_full_unstemmed Hypercoagulability in hereditary hemorrhagic telangiectasia with epilepsy
title_short Hypercoagulability in hereditary hemorrhagic telangiectasia with epilepsy
title_sort hypercoagulability in hereditary hemorrhagic telangiectasia with epilepsy
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4481800/
https://www.ncbi.nlm.nih.gov/pubmed/26167029
http://dx.doi.org/10.4103/0976-3147.158791
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