02134nas a2200193   4500008003900000245012300039210006900162260000800231490000700239520153500246100001701781700001101798700002401809700001701833700001101850700001501861700001501876856004901891       2018                          d00aComplications of a severe autoimmune hemolytic anemia crisis: transfusional iron overload and gangrenous cholecystitis0 aComplications of a severe autoimmune hemolytic anemia crisis tra  cDec0 v583 aBACKGROUND:

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.1 aMenchetti, I1 aLin, Y1 aCserti-Gazdewich, C1 aGoldstein, J1 aLaw, C1 aLazarus, A1 aCallum, JL  uhttps://www.notifylibrary.org/reference/4742