Hereditary hemorrhagic telangiectasia
ICD-10 I78.0 · ICD-11 LA90.00

Managing Recurrent Epistaxis in Hereditary Hemorrhagic Telangiectasia

Recurrent epistaxis is the most common and often most burdensome manifestation of HHT, occurring in over 90% of adults with the condition.

Clinical Scenario

This protocol addresses patients with confirmed hereditary hemorrhagic telangiectasia experiencing recurrent epistaxis — a hallmark feature that frequently requires a structured, escalating treatment strategy.

Treatment Approach

When standard local and topical measures have not adequately controlled epistaxis, the protocol includes systemic antiangiogenic therapy as a recommended option, alongside certain procedural alternatives.

Full agent selection, dosing schedule, sequencing, and procedural criteria are in the complete protocol.

Instant Access to Structured Evidence-Based Regimens
References
DOI: 10.1182/blood.2020008739
Recurrent and often severe epistaxis is the most common manifestation of HHT, occurring in >90% of adults.
On the strength of several relatively small studies available at the time of the guidelines conference, systemic antiangiogenic therapy is now recommended as an option for managing epistaxis that has failed to respond to moisturizing topical therapies, oral tranexamic acid, and/or local procedural ablative therapies.
Risks and benefits of antiangiogenic medications should be considered, as well as alternatives, such as septodermoplasty and nasal closure, in these patients.
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