Treatment of Moderate or Severe HHT-Related Gastrointestinal Bleeding
When gastrointestinal bleeding in hereditary hemorrhagic telangiectasia (HHT) reaches moderate or severe severity — defined by the need for intravenous iron supplementation or red cell transfusion — a specific systemic treatment approach is indicated.
Clinical Scenario
Moderate HHT-related GI bleeding applies to patients who can meet their hemoglobin goals with IV iron treatment.
Severe HHT-related GI bleeding applies to patients who do not meet hemoglobin goals despite adequate iron replacement, or who require blood transfusions.
Both groups require escalation to systemic therapy.
Treatment Goals
The primary goal is a clinically meaningful rise in hemoglobin — a mean increase of approximately 3–4 g/dL — along with substantial reductions in red cell transfusion and iron infusion requirements.
Approach overview: Expert guidance supports the use of systemic antiangiogenic therapy for patients in this group. The complete structured protocol — including agent selection, induction and maintenance sequencing, and individualised management considerations — is available via the link below.
References
DOI: 10.1182/blood.2020008739
- Moderate HHT-related GI bleeding: patient who meets their hemoglobin goals* with IV iron treatment.
- Severe HHT-related GI bleeding: patient who does not meet their hemoglobin goals* despite adequate iron replacement or requires blood transfusions.
- The expert panel recommends that clinicians consider treatment of moderate to severe HHT-related GI bleeding with intravenous bevacizumab or other systemic antiangiogenic therapy.
- Systemic bevacizumab is recommended for patients with moderate or severe GI bleeding (those requiring intravenous iron or red cell transfusion) on the basis of substantial improvements in mean hemoglobin (3-4 g/dL increase), red cell transfusion (>80% reduction), and iron infusion (>70% reduction) described in single-center retrospective cohort studies.
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