Hereditary hemorrhagic telangiectasia is an inherited autosomal dominant disease presenting with recurrent bleeding episodes and iron deficiency anemia due to vascular malformations. Hereditary hemorrhagic telangiectasia is associated with an increased risk of stroke, gastrointestinal bleeding and pulmonary hypertension and life expectancy is significantly reduced. Excess vascular endothelial growth factor (VEGF) plays a key role in the pathophysiology of the disease.
Here we report about a male patient with hereditary hemorrhagic telangiectasia presenting with pulmonary and central nervous system involvement experiencing repetitive nosebleeds, necessitating frequent local cauterization and transfusion of more than 100 units of packed red blood cells. After initiation of temporary therapy with the anti-VEGF antibody bevacizumab at a dosage of 1 mg/kg body weight every 2 weeks, the nose bleeding episodes and the epistaxis severity score significantly decreased and long-lasting transfusion independence was achieved. Reinitiation of low-dose bevacizumab after relapse again proved effective without any documented therapy-related adverse events. In comparison to other reported anti-VEGF antibody protocols in hereditary hemorrhagic telangiectasia, our treatment approach proved to be cost-efficient.
Intermittent low-dose therapy with bevacizumab represents an effective and cost-efficient treatment option for transfusion-dependent patients with hereditary hemorrhagic telangiectasia.
Thompson AB, Ross DA, Berard P, Figueroa-Bodine J, Livada N, Richer SL. Very low dose bevacizumab for the treatment of epistaxis in patients with hereditary hemorrhagic telangiectasia. Allergy Rhinol (Providence). 2014;5(2):91–5. CrossRef
Zhang J, Cai Y, Hu H, Ling J, Xiao J, Deng Y. Bevacizumab, Aflibercept or Ramucirmab combined with chemotherapy as second-line treatment for metastatic colorectal cancer following progression with Bevacizumab in first-line therapy: A systematic review and indirect comparison. J Clin Oncol. 2015;33(Suppl):abstr e14601.
Intermittent low-dose bevacizumab in hereditary hemorrhagic telangiectasia
A case report
MD Richard Greil
- Springer Vienna
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