Life-threatening delayed hyperhemolytic transfusion reaction in a patient with sickle cell disease: effective treatment with eculizumab followed by rituximab.

Abstract

BACKGROUND:

Hyperhemolysis in sickle cell disease is a rare and potentially life-threatening complication of transfusion.

STUDY DESIGN AND METHODS:

In this article we report a case of delayed hemolytic transfusion reaction with resultant hyperhemolysis triggered by an anti-IH autoantibody with alloantibody behavior.

RESULTS:

The anti-IH was reactive at room temperature as well as 37 °C, but only weakly reactive with autologous red blood cells. Initial cold agglutinin titer was 512. The profound, life-threatening, intravascular hemolysis was rapidly and dramatically reduced with the Complement 5 (C5) inhibitory antibody, eculizumab. The auto/allo cold agglutinin was subsequently suppressed with rituximab treatment.

CONCLUSIONS:

Eculizumab, a potent C5 inhibitory antibody, can be a rapid and effective therapy for hyperhemolytic transfusion reactions when given in a sufficient dose to fully block the activation of complement C5.

• Authors: Mark Boonyasampant, Ilene C Weitz, Brian Kay, Chaiyaporn Boonchalermvichian, Howard A Liebman, Ira A Shulman
• Journal: Transfusion (Transfusion) Vol. 55 Issue 10 Pg. 2398-403 (Oct 2015) ISSN: 1537-2995 [Electronic] United States
• PMID: 25989361 (Publication Type: Case Reports, Journal Article)
• Copyright: © 2015 AABB.

Chemical References

• Antibodies, Monoclonal, Humanized
• Isoantibodies
• Rituximab
• eculizumab

Topics

• Adult
• Anemia, Sickle Cell (blood, therapy)
• Antibodies, Monoclonal, Humanized (administration & dosage)
• Blood Group Incompatibility (drug therapy)
• Female
• Hemolysis (drug effects)
• Humans
• Isoantibodies (blood)
• Rituximab (administration & dosage)
• Transfusion Reaction