Severe autoimmune hemolytic anemia associated with IgM warm auto-antibodies in primary Sjögren's syndrome.
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.
Shinoda
Koichiro
K
First Department of Internal Medicine, University of Toyama, Toyama, Japan.
Taki
Hirofumi
H
Hounoki
Hiroyuki
H
Ogawa
Reina
R
Sugiyama
Eiji
E
Tobe
Kazuyuki
K
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
D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D007075:Immunoglobulin M; D011239:Prednisolone