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When a transfusion in an emergency service is not really urgent: hyperhaemolysis syndrome in a child with sickle cell disease
  1. Sara Chinchilla Langeber1,
  2. Marta Pilar Osuna Marco1,
  3. María Benedit2,
  4. Áurea Cervera Bravo1
  1. 1Department of Paediatrics, Hospital Universitario de Mostoles, Mostoles, Spain
  2. 2Department of Paediatrics, Hospital Puerta del Sur, Mostoles, Spain
  1. Correspondence to Dr Sara Chinchilla Langeber, s.chinchillalangeber{at}


A 13-month-old boy with sickle cell disease (SCD) from Equatorial Guinea, who had recently arrived in Spain, presented with fever. He had suffered from malaria and had received a blood transfusion. Following physical examination and complementary tests, intravenous antibiotics and a red blood cell (RBC) transfusion were administered. Soon after a second transfusion 5 days later, the haemoglobin level fell below pretransfusion levels, together with reticulocytopenia, and haematuria—the so-called hyperhaemolysis syndrome—requiring intensive care and treatment with intravenous immunoglobulins and corticosteroids, with resolution of the complication. We want to emphasise the importance of suspecting this rare, though severe complication that can appear after any RBC transfusion especially in patients with SCD, as the clinical syndrome can simulate other more common complications of these patients and a further transfusion is contraindicated. There is no standardised treatment, but intravenous immunoglobulin and corticosteroids are usually effective.

  • haematology (drugs and medicines)
  • paediatrics (drugs and medicines)
  • haematology (incl blood transfusion)

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  • Contributors SCL: planning, conception, reporting, acquisition of data, design and final drafting of the article. MPOM: acquisition of data, critical review, reporting and bibliography search. MB: critical review and English style. ÁCB: planning, conception, conduct and contribution to the final drafting of the article.

  • Funding This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent Parental/guardian consent obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.