Non-Deletional Haemoglobin H Disease: What to Do When the Blood Transfusion Programme Does Not Achieve the Haemoglobin Target

Clinical Scenario

Non-deletional haemoglobin H (HbH) disease presents with diverse clinical phenotypes across affected individuals. This protocol addresses management in patients who have already been established on a structured blood transfusion programme — or have undergone splenectomy in selected circumstances — but have not reached the intended haematological response.

Prior Treatment & Failure Condition

The preceding management step was a regular blood transfusion programme (or, in eligible patients aged over 5 years with limited transfusion access, hypersplenism, or massive splenomegaly: splenectomy). The goal of that line — maintaining a pre-transfusion haemoglobin of 80–90 g/L — was not achieved. Non-achievement of this target is the trigger for escalation to this protocol.

Next Step — Partial Overview

Following failure of the transfusion-based approach to reach haemoglobin targets, the protocol introduces iron chelation therapy as the next management step, to be initiated on the basis of specific iron-status criteria. The complete thresholds, monitoring parameters, and sequencing of this approach are detailed in the full protocol.

Instant Access to Structured Evidence-Based Regimens

References

Clinical phenotypes are diverse among affected individuals with non-deletional haemoglobin H (HbH) disease (--/αTα).

Iron chelation should be started if LIC >5 mg/g dry weight or ferritin >500 ng/mL.

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