Iron deficiency anemia is a common and clinically significant complication of hereditary hemorrhagic telangiectasia. When initial oral iron therapy fails to meet its targets, a structured next-line approach is needed.
Iron deficiency anemia in HHT produces typical symptoms — fatigue, reduced exercise tolerance, pica, restless leg syndrome, and hair loss — and can considerably worsen symptoms of high-output cardiac failure in patients with significant liver AVMs.
The expert panel recommends iron replacement for treatment of iron deficiency and anemia in this population.
Initial therapy with oral iron is the first step in managing iron deficiency anemia in HHT. The treatment goal for that line is a hemoglobin rise of ≥1.0 g/dL and normalization of ferritin and transferrin saturation within one month.
When oral iron is ineffective, inadequately absorbed, or not tolerated — and those targets are not reached — escalation to the next protocol is indicated.
The protocol at this stage involves intravenous iron replacement — one approach for patients in whom oral iron has not been effective, is not absorbed, or is not tolerated.
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.
Intravenous iron replacement for patients in whom oral is not effective, not absorbed or not tolerated, or presenting with severe anemia.
IV iron dose can be guided by total iron deficit (Ganzoni formula) or a total empiric dose of 1 gram can be provided, with interval reassessment.
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