Status:
Ready to upload
Record number:
1651
Adverse Occurrence type:
MPHO Type:
Estimated frequency:
Case report, rare
Time to detection:
2 weeks
Alerting signals, symptoms, evidence of occurrence:
A sickle cell patient presented with severe fatigue, worsening jaundice, and total body pain. Physical examination was notable for scleral icterus and pale conjunctivae. One day after admission, she had a fever of 39°C and her oxygen saturation dropped to 85% on 10 L of oxygen using a non rebreather mask. Hb precipitously decreased to 3.8 g/dL (from 7.4 g/dL) with laboratory values suggestive of acute hemolysis with LDH of more than 5000 IU/L, total bilirubin of 9.8 mg/dL, and haptoglobin of less than 20 mg/dL. Acute kidney injury was also present with serum creatinine increasing to 1.29 mg/dL from 0.77 mg/ dL on admission.
Demonstration of imputability or root cause:
The antibody screen revealed the presence of a panagglutinin that reacted with all panel cells with 4+ strength. The direct antiglobulin test (DAT) by tube method was weakly positive for C3 only. Anti-IH was identified in the patient’s serum that was reactive at room temperature and 37C by strict prewarm technique.
Imputability grade:
3 Definite/Certain/Proven
Groups audience:
Keywords:
Suggest new keywords:
hyperhemolysis, red cell transfusion, scleral icterus, pale conjunctivae, fatigue, worsening jaundice, total body pain, Eculizumab, panagglutinin, DAT,
Reference attachment:
Suggest references:
Boonyasampant, M., Weitz, I. C., Kay, B., Boonchalermvichian, C., Liebman, H. A. and Shulman, I. A.(2015) Life-threatening delayed hyperhemolytic transfusion reaction in a patient with sickle cell disease: effective treatment with eculizumab followed by rituximab. Transfusion doi:10.1111/trf.13144
Expert comments for publication:
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.