Severe Recurrent Gastrointestinal Bleeding Leading to the Diagnosis of Hereditary Hemorrhagic Telangiectasia: Clinical Response to Systemic Bevacizumab in a Case Report and Narrative Review.

Hereditary hemorrhagic telangiectasia (HHT), or Rendu-Osler-Weber disease, is an inherited vascular disorder that may cause severe gastrointestinal bleeding, particularly at an advanced age. The management of diffuse and recurrent gastrointestinal involvement may remain challenging despite repeated endoscopic treatments. We report the case of a 62-year-old woman with recurrent epistaxis since childhood, nasal and cutaneous telangiectases, and severe recurrent gastrointestinal bleeding related to multiple gastric, duodenal, jejunal, and more distal small-bowel telangiectases. A definite diagnosis of HHT was established based on the presence of three Curaçao criteria. During the two years preceding systemic therapy, the patient required 11 hospitalizations, repeated packed red blood cell transfusions, intravenous iron infusions, and 13 sessions of argon plasma coagulation. Intravenous bevacizumab was initiated at a dose of 5 mg/kg every 15 days for six induction cycles, followed by 5 mg/kg every four weeks as maintenance therapy. During one year of follow-up, the patient remained free of gastrointestinal bleeding for the first six months. The first recurrence occurred during the seventh month. Three separate hospitalizations for recurrent bleeding were observed during the year following bevacizumab initiation, compared with 11 during the preceding two years. The two most recent episodes were associated with less severe anemia than the most severe episodes before treatment. Qualitative endoscopic improvement, with an apparent reduction in the number and prominence of antral telangiectases, was also observed. No significant bevacizumab-related adverse events were identified. In this patient with severe HHT-related gastrointestinal involvement, systemic bevacizumab was associated with a clinically meaningful reduction in the overall bleeding burden. This case highlights the importance of considering HHT in patients with multiple or recurrent gastrointestinal telangiectases associated with epistaxis and mucocutaneous telangiectases.