Treatment of hereditary haemorrhagic telangiectasia with bevacizumab – what more needs to be known?
Introduction: Hereditary haemorrhagic telangiectasia (HHT) is a rare multi-organ vascular disease. It is characterised by mucocutaneous telangiectasia, epistaxis, and arteriovenous malformations. Some 70% of patients with HHT are thought to have issues with gastrointestinal (GI) bleeding. Traditiona...
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| Format: | Article |
| Language: | English |
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SMC MEDIA SRL
2023-12-01
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| Series: | European Journal of Case Reports in Internal Medicine |
| Subjects: | |
| Online Access: | https://www.ejcrim.com/index.php/EJCRIM/article/view/4219 |
| _version_ | 1827386544286072832 |
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| author | Edmond Morrissey Daniel Schmidt-Martin Martin Buckley |
| author_facet | Edmond Morrissey Daniel Schmidt-Martin Martin Buckley |
| author_sort | Edmond Morrissey |
| collection | DOAJ |
| description | Introduction: Hereditary haemorrhagic telangiectasia (HHT) is a rare multi-organ vascular disease. It is characterised by mucocutaneous telangiectasia, epistaxis, and arteriovenous malformations. Some 70% of patients with HHT are thought to have issues with gastrointestinal (GI) bleeding. Traditional management of GI bleeding in HHT includes monitoring for iron deficiency anaemia, iron replacement, antifibrinolytic therapy and control of identifiable bleeding sites with argon photocoagulation during gastrointestinal endoscopy. Blood transfusion may also be required.
Case description: Our case describes a man in his 40s with confirmed HHT, with transfusion-dependent anaemia secondary to GI bleeding. He was commenced on fortnightly bevacizumab (5 mg/kg) for 12 weeks in an attempt to reduce his blood transfusion requirement and manage his anaemia. In the months prior to starting bevacizumab, our patient’s transfusion requirement ranged from 3–5 units of packed red cells per month to maintain an Hb >8 g/dl. He had a marked improvement in his symptoms within the first month of treatment and did not require any further blood transfusion during the three months of treatment. He was given one further IV iron infusion in the final month of his 3-month bevacizumab treatment and did not experience any adverse side effects from bevacizumab.
Discussion: HHT results from alterations to genes which encode proteins involved in blood vessel formation. This provides the rationale for using anti VEGF drugs such as bevacizumab. Current evidence for this treatment approach is limited.
Conclusion: Bevacizumab can be an effective treatment option in patients with HHT refractory to traditional management. |
| first_indexed | 2024-03-08T15:47:51Z |
| format | Article |
| id | doaj.art-89ddd14d36fd4c788dfd3d8f05dc9730 |
| institution | Directory Open Access Journal |
| issn | 2284-2594 |
| language | English |
| last_indexed | 2024-03-08T15:47:51Z |
| publishDate | 2023-12-01 |
| publisher | SMC MEDIA SRL |
| record_format | Article |
| series | European Journal of Case Reports in Internal Medicine |
| spelling | doaj.art-89ddd14d36fd4c788dfd3d8f05dc97302024-01-09T09:32:11ZengSMC MEDIA SRLEuropean Journal of Case Reports in Internal Medicine2284-25942023-12-0110.12890/2023_0042193754Treatment of hereditary haemorrhagic telangiectasia with bevacizumab – what more needs to be known?Edmond Morrissey0Daniel Schmidt-Martin1Martin Buckley2Department of Gastroenterology, Mercy University Hospital, Cork, IrelandDepartment of Gastroenterology, Mallow General Hospital, Cork, IrelandDepartment of Gastroenterology, Mercy University Hospital, Cork, IrelandIntroduction: Hereditary haemorrhagic telangiectasia (HHT) is a rare multi-organ vascular disease. It is characterised by mucocutaneous telangiectasia, epistaxis, and arteriovenous malformations. Some 70% of patients with HHT are thought to have issues with gastrointestinal (GI) bleeding. Traditional management of GI bleeding in HHT includes monitoring for iron deficiency anaemia, iron replacement, antifibrinolytic therapy and control of identifiable bleeding sites with argon photocoagulation during gastrointestinal endoscopy. Blood transfusion may also be required. Case description: Our case describes a man in his 40s with confirmed HHT, with transfusion-dependent anaemia secondary to GI bleeding. He was commenced on fortnightly bevacizumab (5 mg/kg) for 12 weeks in an attempt to reduce his blood transfusion requirement and manage his anaemia. In the months prior to starting bevacizumab, our patient’s transfusion requirement ranged from 3–5 units of packed red cells per month to maintain an Hb >8 g/dl. He had a marked improvement in his symptoms within the first month of treatment and did not require any further blood transfusion during the three months of treatment. He was given one further IV iron infusion in the final month of his 3-month bevacizumab treatment and did not experience any adverse side effects from bevacizumab. Discussion: HHT results from alterations to genes which encode proteins involved in blood vessel formation. This provides the rationale for using anti VEGF drugs such as bevacizumab. Current evidence for this treatment approach is limited. Conclusion: Bevacizumab can be an effective treatment option in patients with HHT refractory to traditional management.https://www.ejcrim.com/index.php/EJCRIM/article/view/4219bleedinganaemiabevacizumabhereditary haemorrhagic telangiectasiahht |
| spellingShingle | Edmond Morrissey Daniel Schmidt-Martin Martin Buckley Treatment of hereditary haemorrhagic telangiectasia with bevacizumab – what more needs to be known? European Journal of Case Reports in Internal Medicine bleeding anaemia bevacizumab hereditary haemorrhagic telangiectasia hht |
| title | Treatment of hereditary haemorrhagic telangiectasia with bevacizumab – what more needs to be known? |
| title_full | Treatment of hereditary haemorrhagic telangiectasia with bevacizumab – what more needs to be known? |
| title_fullStr | Treatment of hereditary haemorrhagic telangiectasia with bevacizumab – what more needs to be known? |
| title_full_unstemmed | Treatment of hereditary haemorrhagic telangiectasia with bevacizumab – what more needs to be known? |
| title_short | Treatment of hereditary haemorrhagic telangiectasia with bevacizumab – what more needs to be known? |
| title_sort | treatment of hereditary haemorrhagic telangiectasia with bevacizumab what more needs to be known |
| topic | bleeding anaemia bevacizumab hereditary haemorrhagic telangiectasia hht |
| url | https://www.ejcrim.com/index.php/EJCRIM/article/view/4219 |
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