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.

Cytomegalovirus and Epstein-Barr Infections: Prevalence and Impact on Patients with Hematological Diseases

“…Another case-control study observed a high prevalence of EBV-associated gastric carcinoma in patients with pernicious anemia [ 95 ]. Fadeyi et al (2015) [ 96 ] reported a case of fatal autoimmune hemolytic anemia (AIHA) associated with the reactivation of latent EBV infection in a 31-year-old man. The authors suggested that EBV infection triggered severe hemolysis, which aggravated the AIHA.…”

Efficacy of D– red blood cell transfusion and rituximab therapy in autoimmune hemolytic anemia with anti‐D and panreactive autoantibodies arising after hematopoietic stem cell transplant

“…Known hematopoietic symptoms of cGVHD include autoantibody production associated with AIHA and immune thrombocytopenic purpura . As reported by Fadeyi and colleagues, EBV induced broad warm‐reactive antibodies that exacerbated AIHA. On the other hand, it has also been reported that T‐cell depletion is associated with post‐HSCT AIHA .…”

A Case of Warm Autoimmune Hemolytic Anemia Secondary to Epstein-Barr Virus Infection

“…Our patient had positive warm antibodies, which is unique. An extensive literature review showed only one single report of fatal AIHA due to IgG warm agglutination induced by EBV infection [ 6 ]. We believe that AIHA in our patient is likely due to reactivation of latent EBV infection.…”