Sickle cell disease: when and how to transfuse

Abstract

Blood transfusion remains an important therapeutic intervention in patients with sickle cell disease (SCD), aiming to both increase the oxygen carrying capacity of blood and to reduce the complications of vaso-occlusion. Simple, manual exchange and automated exchange can be effective in reducing the acute and chronic complications of SCD, and the advantages and disadvantages of each methodology mean they all have a role in different situations. Evidence for the role of emergency transfusion in the management of the acute complications of SCD, including acute pain and acute chest syndrome, comes from observational data. Several important randomized controlled trials have shown the efficacy of transfusion in primary and secondary stroke prevention in patients with SCD but, outside these areas, clinical practice lacks a clear evidence base. Evidence for the role of long-term transfusion in the prevention of the non-neurologic chronic complications of SCD comes from analysis of secondary outcomes of these randomized trials and from observational data. In view of the paucity of data, the risks and benefits of transfusion should be fully discussed with patients/families before a long-term transfusion program is commenced. Evidence is only available for the role of preoperative transfusion or for prophylactic transfusion through pregnancy in certain situations, and the role of transfusions outside these situations is discussed. Questions about when and how to transfuse in SCD remain and will need further randomized trials to provide answers. Learning Objectives• To describe the advantages and disadvantages of simple, manual exchange and automated exchange transfusion • To discuss when transfusion should be used to treat the acute and chronic complications of sickle cell disease • To discuss the role of preoperative transfusion and of transfusion during pregnancy in decreasing sickle-related complications

Perioperative care of children with sickle cell disease: A systematic review and clinical recommendations

“…Regarding thresholds, earlier recommendations advocate “no transfusion” prior to surgery or minor risk surgeries, as long as the hemoglobin level was >4 to 5 g/dL . This limit had the tendency to move upwards with time, many authors currently recommending a hemoglobin level ≥9‐10 g/dL, before minor to intermediate risk surgery, whilst some groups still advocate 7 g/dL . A hemoglobin threshold of >10 g/dL is considered by some authors as a target for major surgeries .…”

Choosing Wisely for apheresis

“…RCE allows the transfusion of large quantities of RBCs while simultaneously removing HbS containing RBCs, thereby preventing additional iron accumulation. RCE is a more effective method than simple transfusions to prevent both strokes and the complications of iron overload …”

“…RCE is a more effective method than simple transfusions to prevent both strokes and the complications of iron overload. [18][19][20] 3.5 | Recommendation 4 3.5.1 | Do not routinely monitor coagulation tests during a course of TPE, unless the procedure is performed daily…”

Preoperative transfusion versus no transfusion policy in sickle cell disease patients: a randomized trial

“…Apart from PRBC transfusion, a standardized preoperative care and anesthesia protocol were applied to the studied groups. 7,14,15 On admission, all patients were hydrated with pediatric saline, administered at a rate of 1.5-2 times maintenance. The patients were reviewed by the anesthesiologist.…”