How I manage red cell transfusions in patients with sickle cell disease

Abstract

Sickle cell disease is one of the commonest serious inherited diseases in the world, and red cell transfusion is still one of the few effective treatments for acute and chronic complications. Transfusion corrects anaemia and dilutes out the number of red cells able to cause vaso-occlusion and vascular damage. Urgent red cell transfusions are used to correct acute anaemia, treat acute chest syndrome and patients with acute neurological symptoms. We use elective transfusions preoperatively for moderate risk surgery, and in some pregnant women. There is good evidence for the use of long-term regular transfusions in primary stroke prevention, with the aim of keeping the percentage of sickle haemoglobin below 30%. Long-term transfusions are also used in secondary stroke prevention, and the management of progressive organ damage, including renal impairment and pulmonary hypertension. Blood needs to be matched for ABO, RH and Kell, although alloantibodies may still develop and require more careful, extended cross-matching. Delayed haemolytic transfusion reactions are relatively common, difficult to diagnose and manage, and potentially fatal.

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

“…The use of preoperative transfusion is a source of significant controversy. Case‐by‐case decision‐making is the standard of care in the majority of reports . Prospective clinical studies on this question are presented in Table (all primary research in Table S2).…”

“…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 .…”

“…Some authors advocate for preoperative transfusion without giving a threshold, despite evidence from the abovementioned RCTs. Exchange transfusions (manual and automated) for a leve of HbS <30% is recommended for high risk surgery either preoperatively, or on cardiopulmonary bypass . In cardiac pulmonary bypass, some authors suggest a stricter HbS limit of <5% .…”

Evaluation of the anti-sickling activity of <i>Newbouldia laevis</i> P. Beauv extracts

“…According to Penkert et al (2018) treatment with hydroxyurea to reduce the risk of stroke, acute thoracic syndrome and also reduce inflammatory molecules remains expensive for most patients. Blood transfusion is also proposed with some measures that are difficult to implement in our developing countries (Rees et al, 2018). For these reasons, sickle cell patients in sub-Saharan Africa resort to medicinal plants that have proved their effectiveness.…”

Antisickling Activity Evaluation of Fractions Obtained From Whole Extracts of Newbouldia Laevis P. Beauv (Bignoniaceae)

“…Treatment with hydroxyurea to reduce the risk of stroke, acute thoracic syndrome and also reduce inflammatory molecules [5] remains expensive for most patients. Blood transfusion is also proposed with some measures that are difficult to implement in our developing countries [6]. For these reasons, sickle cell patients in sub-Saharan Africa resort to medicinal plants that have proved their effectiveness.…”