Treatment of Hemoglobin H Disease – Non-Deletional Form (--/αTα)
Clinical Scenario
This protocol addresses the management of Haemoglobin H (HbH) disease in its non-deletional form,
defined by the presence of a non-deletional α-globin gene mutation (genotype --/αTα).
Clinical phenotypes are diverse among affected individuals with non-deletional HbH disease.
Importantly, clinical symptoms in the non-deletional form are generally more severe than
those seen in deletional forms of HbH disease.
Non-deletional HbH disease typically carries a more severe clinical course than its deletional counterpart.
Treatment Approach & Goals
Management of symptomatic anaemia in this setting involves an on-demand red blood cell support strategy,
guided by haemoglobin thresholds and the presence of anaemia-related symptoms.
The structured protocol specifies the precise trigger thresholds, target haemoglobin range (80–90 g/L),
and the full transfusion regimen — including blood product requirements. Access the complete protocol below.
References
- Clinical phenotypes are diverse among affected individuals with non-deletional haemoglobin H (HbH) disease (--/αTα).
- Clinical symptoms of non-deletional HbH are generally more severe than those of deletional forms.
- This intervention is recommended when the haemoglobin level drops below 70 g/L or when there is accompanying symptoms of anaemia, with an aim to restore Hb to 80–90 g/L.
- Leucocyte-depleted red blood cells should be administered at a volume of 10–15 ml/kg (1–2 units for adults) one or more times based on the severity of anaemia.
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