Educational draft · awaiting clinical reviewUse Rapid for revision, not patient-care decisions. Check current national and local guidance and the BNF or BNFC before acting.
2 min synopsisUK scopeSources checked 27 Aug 2026Clinical review pending
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Escalate
Fever with a central line or after splenectomy, acute heart failure or arrhythmia in severe iron loading, a major transfusion reaction, or symptomatic profound anaemia needs immediate specialist care. Stop a transfusion for suspected serious reaction, maintain venous access with saline, check identity and notify the transfusion laboratory. Deferiprone-associated fever or sore throat requires immediate cessation and urgent full blood count because agranulocytosis can be rapidly fatal.
Synopsis
Deliver phenotype-matched regular transfusion, prevent ineffective-erythropoiesis complications, quantify tissue iron and tailor chelation while protecting endocrine, hepatic, cardiac and reproductive health.
Transfusion-dependent beta-thalassaemia requires regular red cells to sustain growth, activity and organ oxygenation and suppress harmful marrow expansion and extramedullary haematopoiesis.
Before the first elective transfusion, record an extended red-cell phenotype or genotype and antibody screen; use leucocyte-depleted, appropriately antigen-matched units through the specialist programme.
Pre-transfusion haemoglobin targets and intervals are individualised, commonly maintaining about 90–105 g/L, with higher targets for selected cardiac or extramedullary indications.
Key red flags
Chelator emergency
Fever or painful throat while taking deferiprone requires stopping the drug and obtaining an urgent neutrophil count.
Investigation priorities
01
Pre-transfusion full count and antibody screenFirst step
A patient meets specialist criteria for transfusion-dependent thalassaemia care.
Record genotype, extended red-cell antigens, antibody and reaction history and obtain baseline viral, iron and organ assessments before repeated exposure.
Give leucocyte-depleted, appropriately antigen-matched red cells, commonly 10–15 mL/kg every 2–4 weeks, to maintain a pre-transfusion haemoglobin of about 90–105 g/L; use a higher specialist target only for a defined cardiac or extramedullary indication.
Key medicines
Leucocyte-depleted red cellsA common maintenance prescription is 10–15 mL/kg every 2–4 weeks, adjusted to maintain pre-transfusion haemoglobin around 90–105 g/L and suppress ineffective erythropoiesis. Use appropriately antigen-matched units and calculate actual annual volume per kilogram.
DeferasiroxFor film-coated tablets, a common starting dose with frequent transfusion is 14 mg/kg orally once daily; 7 mg/kg may suit a lower iron input and 21 mg/kg a high input needing reduction. Adjust by 3.5–7 mg/kg every 3–6 months to ferritin and MRI response, up to 28 mg/kg/day. Dispersible-tablet doses are about 30% higher and are not interchangeable.
National guidance is shown before implementation-dependent detail. Typical adult dose examples remain subject to patient factors, contraindications and the live BNF or specialist protocol. Source check completed 27 Aug 2026; clinical approval remains outstanding.