Description:
In patients with sickle cell disease (SCD), transfusion, by modifying the hemoglobin (Hb)A/HbS red blood cell (RBC) ratio, remains a major treatment to prevent or treat acute vaso-occlusive or chronic complications. Dr. Anoosha Habibi will discuss the indications for transfusion during pregnancy. Pregnancy is a high-risk situation for both mother and child. Recent studies and meta-analyses tend to favor transfusion during pregnancy, but no randomized studies have been conducted to date. The frequency of transfusion complications in pregnant women, such as post-transfusion hyperhemolysis, makes decision-making difficult. As recommended by the ASH guidelines, the decision should be made case-by-case, based on each patient's transfusion history and complications associated with SCD. Dr. Shannon Kelly will review the different transfusion methods that are available when transfusion is indicated : simple RBC transfusion or RBC exchange which can be automated or manual. The role of these different methods depends not only on local resources, but also on the transfusion indication and a number of patient-dependent parameters. Risks and benefits will be discussed. Dr. France Pirenne will define the patients at risk for transfusion: they are those who previously developed RBC antibodies and/or post-transfusion hyperhemolysis. In these situations, prevention of additional reactions with specific transfusion protocols is necessary, immunosupressive therapy is frequently given, and close transfusion monitoring is implemented. In patients with severe disease, hematopoietic stem cell transplantation may be indicated. However, transfusion is also required in this context, and its management is complex as transfusion risks must be taken into account.