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Ready to upload
Record number:
1980
Adverse Occurrence type:
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Estimated frequency:
Evans syndrome is a rare autoimmune disorder that is defined by the simultaneous or sequential presence of two or more cytopenias without an obvious underlying precipitating cause. Autoimmune hemolytic anemia (AIHA) is one of the principal features of Evans syndrome and can vary greatly in severity and symptoms due to significant clinical heterogeneity. Due to these differing presentations, the severity of the resulting anemia varies, ranging from self-limiting episodes managed with prednisone alone to life-threatening hemolytic crises. Aggressive hemolysis is typically managed via corticosteroids. Red blood cell (RBC) transfusions, intravenous immunoglobulin (IVIG) and immunosuppressive therpies, with each treatment presenting its own array of adverse effects. Iron overload after a severe AIHA crisis is not well reported in the literature.
Time to detection:
A 66-year-old male patient with a 17-year history of Evans syndrome presented with a fulminant AIHA. He required the transfusion of 20 units of RBCs while awaiting response to aggressive immunosuppresive therapy. He achieved a complete hematologic response. At 12 weeks post-AIHA crisis and after 20 units of RBCs, his ferritin level was 4933 ug/L (normal range: 20-400 ug/L) with an iron saturation of 64%.
Alerting signals, symptoms, evidence of occurrence:
The transfusional iron overload required 16 phlebotomies to reduce his ferritin level from 4933 ug/L to 326 ug/L, with phlebotomies ongoing every 2 weeks to achieve a ferritin level of less than 100 ug/L. In addition, the course was complicated by acute cholecystitis requiring laparoscopic cholecystectomy.
Demonstration of imputability or root cause:
The patient tested negative for HFE mutations including the common C282Y and H63D mutations and had never taken oral iron suplements
Imputability grade:
3 Definite/Certain/Proven
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Reference attachment:
Suggest references:
Complications of a severe autoimmune hemolytic anemia crisis: transfusional iron overload and gangrenous cholecystitis. Transfusion. 2018 Oct 6
Expert comments for publication:
This case demonstrates some of the potential adverse events that can occur as a result of an AIHA crisis in Evans syndrome, particularly transfusional iron overload and acute cholecystitis.