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Delayed haemolytic transfusion reaction in paediatric patients with sickle cell disease: A retrospective study in a
Marica Rossi1, France Pirenne2, Enora Le Roux3
1Sickle Cell Disease Center, Hematology Unit, Robert Debré Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.
Insights
Delayed haemolytic transfusion reactions (DHTR) in children with sickle cell disease are serious but manageable. This study details 41 cases, highlighting common symptoms, complications like acute chest syndrome, and effective treatments such as corticosteroids.
Area of Science:
- Pediatric Hematology
- Transfusion Medicine
- Sickle Cell Disease Management
Background:
- Delayed haemolytic transfusion reaction (DHTR) is a severe complication post-red blood cell transfusion.
- Data on DHTR in pediatric sickle cell disease (SCD) patients is limited.
- Understanding DHTR in children is crucial for managing SCD complications.
Purpose of the Study:
- To analyze the characteristics, clinical presentation, and outcomes of DHTR in children with sickle cell disease.
- To identify risk factors and complications associated with DHTR in this pediatric population.
- To inform the development of management guidelines for DHTR in children with SCD.
Main Methods:
- Retrospective analysis of 41 pediatric cases of DHTR.
- Data collected from a French university hospital between 2006 and 2020.
- Review of patient demographics, transfusion history, clinical manifestations, laboratory findings, and treatment outcomes.
Main Results:
- DHTR occurred at a median age of 10.5 years, often following transfusions for acute events.
- Common findings included profound anemia, low reticulocyte counts, and elevated LDH.
- Significant complications such as acute chest syndrome and kidney failure occurred in 51% of patients; no deaths were reported.
Conclusions:
- DHTR presents unique challenges in pediatric sickle cell disease patients.
- Early recognition and management, including corticosteroids, can mitigate complications.
- Further research is needed to establish specific clinical guidelines for DHTR in children.
Abstract:
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.
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