Atrium Health Huntersville, North Carolina, United States
Background/Case Studies: Patients with sickle cell disease (SCD) frequently require blood transfusions, which can lead to a higher incidence of hemolytic transfusion reactions (HTRs). This case study highlights a rare occurrence of an acute HTR linked to the presence of antibodies to low incidence antigens, He and Goa, which may not be identified with routine antibody screening.
Study
Design/Methods: A 34-year-old female with a history of SCD, presented to the emergency department with back pain, rib pain, dysuria, hematuria, cough and emesis. She had no history of antibodies and a negative antibody screen; therefore, electronically crossmatched units were prepared. After receiving one unit of red blood cells, intended to improve her hemoglobin levels, she experienced signs of hemolysis, including significantly elevated bilirubin levels, dark urine, and tachycardia. Laboratory tests indicated a drop in hemoglobin from 6.0 g/dL to 4.9 g/dL and an increase in lactate dehydrogenase (LDH), further meeting the diagnostic criteria of an acute HTR.
Results/Findings: Retrospective serologic testing showed the unit implicated in the reaction to be incompatible with Indirect Antiglobulin Testing (IAT) in gel with the pre transfusion specimen. Further testing identified the presence of anti-He. Monocyte Monolayer Assay (MMA) results indicated an additional antibody, later confirmed as anti-Goa. While little is known about anti-He, MMA demonstrated that it is clinically significant when testing of the transfused unit yielded elevated values. This evidence further supported the clinical relevance of both antibodies involved in the HTR.
The patient's management included supportive care with intravenous fluids, pain control, IVIG corticosteroids and eculizumab to dampen the immune response. Although her hemoglobin decreased to a nadir of 2.4 g/dL, there were no further transfusions. Her vital signs were closely monitored throughout her hospitalization. Over the next several weeks, her hemoglobin levels returned to baseline, and she demonstrated significant clinical improvement.
Conclusions: This case questions the safety of electronic crossmatch in SCD patients, specifically those with rare antibodies like anti-He or anti-Goa. Notably, pre-transfusion antibody screening was negative, and the patient qualified for electronic crossmatching, which contributed to the adverse reaction. Implementing enhanced screening protocols and refined transfusion strategies is crucial to safeguarding patient safety and improving clinical outcomes in this vulnerable population. Further research is needed to enhance transfusion practices and formulate guidelines for managing patients with rare antibodies effectively.
