Complications of a severe autoimmune hemolytic anemia crisis: transfusional iron overload and gangrenous cholecystitis

TitleComplications of a severe autoimmune hemolytic anemia crisis: transfusional iron overload and gangrenous cholecystitis
Publication TypeJournal Article
Year of Publication2018
AuthorsMenchetti I, Lin Y, Cserti-Gazdewich C, Goldstein J, Law C, Lazarus A, Callum JL
Volume58
Issue12
Date PublishedDec
Abstract

BACKGROUND:

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. Evans syndrome usually follows a chronic relapsing and remitting course and is quite rare, making it difficult to evaluate in clinical studies.
CASE REPORT:

A 66-year-old male patient with a 17-year history of Evans syndrome presented with fulminant autoimmune hemolytic anemia (AIHA). He presented with a markedly elevated C-reactive protein (CRP; 46 mg/L [normal, 0-5 mg/L]) before onset of a decrease in hemoglobin. He required the transfusion of 20 units of red blood cells while awaiting response to aggressive immunosuppressive therapy including high-dose corticosteroids, intravenous immunoglobin therapy, and rituximab. He achieved a complete hematologic response.
RESULTS:

His postdischarge course was complicated by acute cholecystitis requiring laparoscopic cholecystectomy. In addition, his transfusional iron overload requiring 16 phlebotomies to reduce his ferritin level from 4933 μg/L to 326 μg/L, with phlebotomies ongoing every 2 weeks to achieve a ferritin level of less than 100 μg/L.
CONCLUSION:

Neither transfusional iron overload nor acute cholecystitis are well-recognized complications of a severe episode of AIHA. An elevated CRP has been recently recognized as an important prognostic marker in patients with immune thrombocytopenic purpura and this case suggests a need to evaluate its utility in AIHA.

DOI10.1111/trf.14888
Alternate JournalTransfusion
Notify Library Reference ID4742

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