title: Fatal warm autoimmune hemolytic anemia resulting from IgM autoagglutinins in an infant with severe combined immunodeficiency.
abstract: Autoimmune diseases are rare in patients with severe combined immunodeficiency (SCID). The authors describe an 11-month-old infant girl with SCID with fatal warm autoimmune hemolytic anemia (AIHA) resulting from IgM autoagglutinins. Serologic evaluation revealed IgM autoantibodies that caused in vitro hemagglutination at 37 degrees C. The patient had clinical evidence of ongoing hemolysis and agglutination despite aggressive treatment. She had three strokes and died 6 weeks after unsuccessful bone marrow transplantation. Autoimmune disease is an unexpected complication of SCID. The presence of warm reactive IgM autoagglutinins in AIHA confers a dismal prognosis.
authors.affiliation: Department of Pediatrics, Mount Sinai Medical Center, New York, NY 10029, USA.
authors.forename: A
authors.initials: A
authors.lastname: Nowak-Wegrzyn
authors.forename: K E
authors.initials: KE
authors.lastname: King
authors.forename: R S
authors.initials: RS
authors.lastname: Shirey
authors.forename: A R
authors.initials: AR
authors.lastname: Chen
authors.forename: C
authors.initials: C
authors.lastname: McDonough
authors.forename: H M
authors.initials: HM
authors.lastname: Lederman
mesh_terms: D000744:Anemia, Hemolytic, Autoimmune / Q000209:etiology* / Q000628:therapy; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies / Q000096:biosynthesis / Q000276:immunology*; D001327:Autoimmune Diseases / Q000209:etiology* / Q000276:immunology; D016026:Bone Marrow Transplantation; D005078:Exchange Transfusion, Whole Blood; D017809:Fatal Outcome; D005260:Female; D006388:Hemagglutinins / Q000096:biosynthesis / Q000276:immunology*; D006801:Humans; D007075:Immunoglobulin M / Q000096:biosynthesis / Q000276:immunology*; D007223:Infant; D011379:Prognosis; D012008:Recurrence; D000069283:Rituximab; D016511:Severe Combined Immunodeficiency / Q000150:complications* / Q000276:immunology / Q000628:therapy; D020521:Stroke / Q000209:etiology; D013696:Temperature; D014184:Transplantation, Homologous
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D006388:Hemagglutinins; D007075:Immunoglobulin M; D000069283:Rituximab