Delayed haemolytic transfusion reaction in paediatric patients with sickle cell disease: A retrospective study in a French national reference centre

Abstract

Summary Delayed haemolytic transfusion reaction (DHTR) is a life‐threatening haemolytic anaemia following red blood cell transfusion in patients with sickle cell disease, with only scarce data in children. We retrospectively analysed 41 cases of DHTR in children treated between 2006 and 2020 in a French university hospital. DHTR manifested at a median age of 10.5 years, symptoms occurred a median of 8 days after transfusion performed for an acute event (63%), before surgery (20%) or in a chronic transfusion programme (17%). In all, 93% of patients had painful crisis. Profound anaemia (median 49 g/L), low reticulocyte count (median 140 ×109/L) and increased lactate dehydrogenase (median 2239 IU/L) were observed. Antibody screening was positive in 51% of patients, and more frequent when there was a history of alloimmunisation. Although no deaths were reported, significant complications occurred in 51% of patients: acute chest syndrome (12 patients), cholestasis (five patients), stroke (two patients) and kidney failure (two patients). A further transfusion was required in 23 patients and corticosteroids were used in 21 to reduce the risk of additional haemolysis. In all, 13 patients subsequently received further transfusions with recurrence of DHTR in only two. The study affords a better overview of DHTR and highlights the need to establish guidelines for its management in children.

Factors associated with adverse outcome among children with sickle cell disease admitted to the pediatric intensive care unit: an observational cohort

“…Finally, it is interesting to note that patients with AO did not differ from the other less severe patients regarding the frequency of transfusion prior to PICU transfer. In fact, with the growing number of patients developing delayed hemolytic transfusion reactions [ 34 , 35 ], this result could be taken into account when evaluating the benefice-risk balance of red blood cell transfusion and other risk factors should be taken into consideration to intensify the patient’s treatment. In our study, HbS level was not significantly different between the two groups.…”