Post‐Transfusion Hyperhaemolysis in a Patient with Sickle Cell Disease: Use of Steroids and Intravenous Immunoglobulin to Prevent Further Red Cell Destruction

Abstract

Delayed haemolytic transfusion reactions (DHTRs) are seen more frequently in patients with sickle cell disease (SCD) than in other groups of patients, and are characterised by a positive direct antiglobulin test and the appearance of previously undetected red blood cell (RBC) alloantibodies in the patient's serum. Recently a syndrome of post-transfusion hyperhaemolysis has been described in children with SCD, characterised by destruction of both autologous and transfused RBCs with negative serological findings: continuation of RBC transfusion exacerbated haemolysis further. We describe a case of life-threatening post-transfusion hyperhaemolysis in an adult patient with SCD in whom severe anaemia necessitated further RBC transfusion, which was successfully performed in conjunction with intravenous immunoglobulin. This approach may be useful in the management of post-transfusion hyperhaemolysis in SCD as well as in the management of severe DHTRs.

Intravenous immune globulin prevents venular vaso-occlusion in sickle cell mice by inhibiting leukocyte adhesion and the interactions between sickle erythrocytes and adherent leukocytes

“…While IVIG was beneficial 3 cases, 20,21 in the fourth case there was a temporal relationship between IVIG administration and the occurrence of a sickle cell crisis. 22 Our data raise the possibility that IVIG may be beneficial in preventing or treating sickle cell vaso-occlusion.…”

Delayed hemolytic transfusion reaction in children with sickle cell disease

“…Previously described patients usually received corticosteroid therapy, 6,7,14 IVIg, 8 or both. 5,9,21 The protocol currently used at our institution involves IVIg infusion.…”

“…Corticosteroid and/or intravenous immunoglobulin (IVIg) therapy has been reported to ensure recovery from DHTR. [5][6][7][8][9]13,14 More recently, two studies reported the use of rituximab for preventing DHTR. 13,15 The pathophysiology of this syndrome remains unclear, especially when there is no detectable antibody.…”

“…These antibodies can cause severe delayed hemolytic transfusion reactions (DHTRs), which present specific features in SCD patients. [5][6][7][8][9] DHTR has been reported to occur in 4-11% of SCD patients given blood transfusions. 6,[10][11][12] DHTRs occur from a few days to two weeks after a blood transfusion and manifest as clinical features of acute hemolysis (hemoglobinuria, jaundice, and pallor) combined with symptoms suggesting severe vaso-occlusive crisis (pain, fever, and sometimes acute chest syndrome).…”

Fatal Delayed Haemolytic Transfusion Reaction and Hyperhaemolysis Syndrome in a Pregnant Woman with Sickle Cell Anaemia

“…The rapid drop in Hb level may be contributed by hyperhaemolysis syndrome. It is regarded as a separate clinical manifestation that can only follow a DHTR [6]. The exact mechanism of hyperhaemolysis syndrome is not well understood.…”