Acute hemolytic transfusion reaction, anti-Sc2

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Adverse Occurrence type: 
MPHO Type: 
Estimated frequency: 
Anti-Sc2 has not been known to cause significant hemolytic transfusion reactions. This report represents the fist case of severe hemolytic transfusion reaction (HTR) caused by anti-Sc2.
Time to detection: 
A nulliparous 26-year-old woman female B-thalassemia major patient, group AB D+ received 75 ml of a single plasma volume-reduced, AB D+, Kell-negative unit, compatible by electronic crossmatch when immediately appeared signs and symptoms of an acute HTR.
Alerting signals, symptoms, evidence of occurrence: 
She rapidly experienced rigors, nausea, abdominal pain and high-grade fever (39.4ºC). Vital signs remained otherwise normal. Laboratory variables were consistent with acute hemolysis. Renal and liver function tests remained normal. Urine analysis was negative for hemoglobinuria. Sepsis investigations were negative.
Demonstration of imputability or root cause: 
A clinical and serologic investigation was performed. The patient was investigated for a red blood cell (RBC) reaction 9 years prior, when she developed symptoms of HTR. The RBC unit was crossmatched by immediate spin due to consistent screen negativity. Full crossmatch found the RBC 1+ incompatible by gel IAT with both pre/post samples, while direct antiglobulin test was negative (pre) and 1+ immunoglobulin IgG positive (post). The antibody remained unidentified and she was committed to gel IAT crossmatch. Two-years later, the specificity to Sc2 was deduced when one RBC unit was found 3+ incompatible. Finally the transfusion reaction reported herein occurred when she received by happenstance RBCs from the same donor who was associated with the remote reaction 9 years earlier. Monocyte monolayer assay (MMA) yielded highly positive phagocytic indices only for Sc2+ RBCs, including the donor's RBCs that triggered the severe HTR.
Imputability grade: 
3 Definite/Certain/Proven
Groups audience: 
Suggest new keywords: 
rigors, nausea, abdominal pain and high-grade fever
IAT, Monocyte monolayer assay (MMA)
low prevalence antigen
Reference attachment: 
Suggest references: 
The first case of severe acute hemolytic transfusion reaction caused by anti-Sc2. Transfusion. Lemay AS, 2018 Oct 9