Transfusion therapy in sickle cell disease

Transfusion in SCD is used either as an emergency measure or as prevention of short and long term complications of SCD (1).

There are 2 main objectives of blood transfusion in SCD:

• to correct anaemia, which will improve the oxygen carrying capacity of blood
• to lower the percentage of HbS relative to HbA in order to treat or prevent the occurrence of painful/vaso-occlusive or sequestration complications (1)

Transfusion in acute medical emergencies can be:

• top up transfusion - in transient red cell aplasia, acute splenic sequestration
• exchange transfusion - in acute stroke, acute chest syndrome, severe sepsis, acute hepatic sequestration, acute multiorgan failure

Indications for elective blood transfusion include:

• for prevention of primary and secondary stroke
• elective surgery
• painful crises in pregnancy (1)

There are several important complications of blood transfusion which may have a negative impact on the long term health of the SCD patients.

• alloimmunisation
• infection - vaccination against hepatitis A and B should be offered to all those on long term transfusion programmes
• iron overload - iron chelation should be started in all patients receiving regular blood transfusion according to standard protocols (1,2)

Reference

1. Chou ST; Transfusion therapy for sickle cell disease: a balancing act. Hematology Am Soc Hematol Educ Program. 2013;2013:439-46
2. Shah FT, Porter JB, Sadasivam N, et al. Guidelines for the monitoring and management of iron overload in patients with haemoglobinopathies and rare anaemias. Br J Haematol. 2022 Jan;196(2):336-50.