Jornal de Distúrbios do Sangue e Transfusão

Abstrato

Fatal Delayed Hemolytic Transfusion Reaction and Hyperhemolysis without Detectable Alloantibodies or Autoantibodies in a Patient with Sickle Cell Disease: A Case Report and Literature Review

Basile Nsimba, Anoosha Habibi, France Pirenne, Pablo Bartolucci, Daniel Tonduangu, Christophe Duvoux, Nicolas De Prost, Maud Marcandetti, Armand Mekontso-Dessap, Frédéric Galactéros, Pascal Morel

Background: Sickle cell disease (SCD) is one of the most commonly inherited diseases worldwide and one of the most frequently occurring genetic disorders in France. Delayed hemolytic transfusion reaction (DHTR) is a classic complication in patients with SCD who undergo blood transfusions, and this condition may lead to hyperhemolysis syndrome (HS). DHTR is described as a transfusion complication and is often associated with anti-RBC antibodies.

Case report: We report the death of a 47-year-old man of Martinican origin, with sickle cell disease (SCD) and no history of alloimmunization, who was admitted to the Sens Medical Center in July 2015 due to a vaso-occlusive crisis (VOC). An antibody screening test based on the gel technique was conducted and no anti-RBC antibodies were detected. The patient was readmitted with a VOC two days after discharge and subsequently developed DHTR/HS after transfusion episodes. He succumbed to acute complications involving severe multiple organ failure after being transferred to Henri-Mondor University Hospital in Creteil. This case report was made available thanks to our Haemovigilance Network.

Conclusion: This case demonstrates the importance of DHTR prevention using national guidelines to carefully assess indications for RBC transfusion in patients with SCD. Early diagnosis is crucial to prevent this life-threatening complication. Special attention should be given to less well-known and less-well monitored patients who may be at high risk for DHTR. In any event, DHTR/HS without detectable anti-RBC antibodies presents a clinical and biological challenge to our understanding of this disorder.