Non-Deletional Haemoglobin H Disease: Next Step When On-Demand Transfusion Has Not Restored Haemoglobin
This page addresses the management of non-deletional haemoglobin H (HbH) disease in patients whose previous on-demand transfusion approach has not consistently achieved the haemoglobin restoration target.
Clinical scenario
Non-deletional HbH disease is characterised by diverse clinical phenotypes among affected individuals. The variability in disease severity means that a reactive, as-needed approach to transfusion may be insufficient to maintain adequate haemoglobin levels in a meaningful subset of patients.
Previous approach — target not reached
The preceding line used on-demand red blood cell transfusion, administered when haemoglobin fell below 70 g/L, with the aim of restoring haemoglobin to 80–90 g/L. When that restoration target is not consistently met, the protocol below is the indicated next step.
Next-line approach (partial overview)
This protocol moves away from reactive, on-demand transfusion toward a scheduled blood transfusion programme. For certain eligible patients, a surgical intervention may additionally be considered as part of the management plan. The clinical goal is a pre-transfusion haemoglobin of 80–90 g/L. The complete protocol — including the selection criteria, schedule, and any supplementary measures — is available via the link below.
References
- Clinical phenotypes are diverse among affected individuals with non-deletional haemoglobin H (HbH) disease (--/αTα).
- Once initiated, blood transfusions should be scheduled, usually every 3 to 6 weeks, with pre-transfusion haemoglobin aimed at a slightly lower level (80–90 g/L) in comparison to that aimed for β-TM.
- Splenectomy can increase Hb levels and decrease or eliminate the need for transfusions in patients with HbH-CS. Splenectomy should be avoided in patients younger than 5 years.
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