title: Severe autoimmune hemolytic anemia associated with IgM warm auto-antibodies in primary Sjögren's syndrome.
abstract: Primary Sjögren's syndrome is an autoimmune disorder involving mainly salivary and lachrymal glands. However, many extraglandular symptoms have also been reported. Although leucocytopenia and lymphocytopenia are frequently observed in hematological disorders, autoimmune hemolytic anemia is rarely reported. We experienced a case of primary Sjögren's syndrome developing severe autoimmune hemolytic anemia. The patient's red blood cells showed spontaneous agglutination in saline at room temperature, and immunoglobulin M (IgM) was detected on the surface of red blood cells by flow cytometry, indicating that autoimmune hemolytic anemia was caused by warm reactive IgM antibodies. Immediate corticosteroid therapy resulted in a dramatic recovery. We report a first case of severe autoimmune hemolytic anemia caused by warm reactive IgM antibodies in primary Sjögren's syndrome.
authors.affiliation: First Department of Internal Medicine, University of Toyama, Toyama, Japan.
authors.forename: Koichiro
authors.initials: K
authors.lastname: Shinoda
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Taki
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Hounoki
authors.forename: Reina
authors.initials: R
authors.lastname: Ogawa
authors.forename: Eiji
authors.initials: E
authors.lastname: Sugiyama
authors.forename: Kazuyuki
authors.initials: K
authors.lastname: Tobe
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000371:Agglutination; D000372:Agglutination Tests; D000744:Anemia, Hemolytic, Autoimmune / Q000097:blood / Q000188:drug therapy / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood*; D004912:Erythrocytes / Q000187:drug effects / Q000276:immunology*; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D007075:Immunoglobulin M / Q000097:blood*; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use; D012720:Severity of Illness Index; D012859:Sjogren's Syndrome / Q000097:blood / Q000150:complications / Q000188:drug therapy / Q000276:immunology*; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D007075:Immunoglobulin M; D011239:Prednisolone