Treatment of Hereditary Hemorrhagic Telangiectasia with Iron Deficiency Anemia
This protocol addresses the management of iron deficiency anemia in patients with hereditary hemorrhagic telangiectasia (HHT), a clinically significant complication that warrants a structured, evidence-based approach.
Iron deficiency anemia in HHT produces typical signs and symptoms — including fatigue, reduced exercise tolerance, pica, restless leg syndrome, and hair loss — and may considerably worsen symptoms of high-output cardiac failure in patients with significant liver arteriovenous malformations.
Treatment Approach
In certain clinical circumstances, red blood cell transfusion may be considered as part of the management strategy. The full decision framework — covering the specific indications and the clinical conditions that guide its use — is detailed in the complete protocol.
References
DOI: 10.1182/blood.2020008739
- The expert panel recommends iron replacement for treatment of iron deficiency and anemia as follows:
- Iron deficiency anemia in HHT results in typical signs and symptoms, including fatigue, reduced exercise tolerance, pica, restless leg syndrome, hair loss, and others, and may considerably worsen symptoms of high-output cardiac failure in patients with significant liver AVMs.
- The expert panel recommends RBC transfusions in the following settings: Hemodynamic instability/shock; Comorbidities that require a higher hemoglobin target; Need to increase the hemoglobin acutely, such as prior to surgery or during pregnancy; Inability to maintain an adequate hemoglobin despite frequent iron infusions.
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