title: Fatal autoimmune hemolytic anemia due to immunoglobulin g autoantibody exacerbated by epstein-barr virus.
abstract: Most cases of autoimmune hemolytic anemia (AIHA) are caused by the production of an autoantibody that targets determinants on red blood cells (RBCs). This autoantibody can be immunoglobulin (Ig) G, IgM, or IgA. Some autoantibodies react optimally at 0° to 4°C (ie, cold agglutinin) and usually are clinically insignificant. High-titer cold agglutinins are associated with IgM autoantibody and complement fixation induced by infectious agents, including the Epstein-Barr virus (EBV). This case report describes a 31-year-old man who had jaundice, a hemoglobin of 6.0 gdL, and was diagnosed with a hemolytic crisis of AIHA. He received a total of 11 RBC transfusions during a 15-hour period without sustained response and later died. The direct antiglobulin test results for this patient were positive, whereas the cold-agglutinin-testing results were negative. We detected EBV DNA in blood via polymerase chain reaction (PCR). We report a rare case of AIHA associated with an IgG autoantibody and exacerbated by EBV infection, causing a fatal hemolytic anemia.
authors.affiliation: Departments of Pathology, Wake Forest University School of Medicine and Wake Forest Baptist Health, Winston-Salem, NC efadeyi@wakehealth.edu.
authors.forename: Emmanuel A
authors.initials: EA
authors.lastname: Fadeyi
authors.affiliation: Wake Forest Baptist Health, Winston-Salem, NC.
authors.forename: Julie H
authors.initials: JH
authors.lastname: Simmons
authors.affiliation: Wake Forest Baptist Health, Winston-Salem, NC.
authors.forename: Mary Rose
authors.initials: MR
authors.lastname: Jones
authors.affiliation: Departments of Pathology, Wake Forest University School of Medicine and Wake Forest Baptist Health, Winston-Salem, NC.
authors.forename: Elizabeth L
authors.initials: EL
authors.lastname: Palavecino
authors.affiliation: Departments of Pathology, Wake Forest University School of Medicine and Wake Forest Baptist Health, Winston-Salem, NC.
authors.forename: Gregory J
authors.initials: GJ
authors.lastname: Pomper
mesh_terms: D000328:Adult; D000744:Anemia, Hemolytic, Autoimmune / Q000276:immunology* / Q000628:therapy / Q000821:virology*; D001323:Autoantibodies / Q000097:blood*; D003450:Cryoglobulins / Q000378:metabolism; D004279:DNA, Viral / Q000097:blood; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D017707:Erythrocyte Transfusion / Q000379:methods; D004854:Herpesvirus 4, Human / Q000235:genetics / Q000472:pathogenicity; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D008297:Male
chemical_list: D001323:Autoantibodies; D003450:Cryoglobulins; D004279:DNA, Viral; D007074:Immunoglobulin G; C029443:cold agglutinins
keywords: EBV; IgG; IgM; autoantibody; direct antiglobulin test; hemolytic anemia; red blood cell transfusions