title: Hemophagocytic syndrome in elderly patients with underlying autoimmune diseases.
abstract: In patients with autoimmune disease-associated hemophagocytic syndrome (AAHS), the clinical features may differ from hemophagocytic syndrome (HPS) of other etiologies, and new criteria for AAHS have been proposed. Since bone marrow (BM) circumstances are changed according to aging, here we reviewed retrospectively our cases with AAHS in elderly patients, including two systemic lupus erythematosus (SLE), three Evans syndrome, one rheumatoid arthritis (RA), one Hashimoto thyroiditis, and one autoimmune pancreatitis. Although only two SLE patients were diagnosed as HPS by the classical criteria, the remaining patients except one RA met the criteria for AAHS. Seven patients except one SLE patient showed good response to therapy and demonstrated positive autoantibodies to blood cells, lower serum ferritin levels, and increased erythroblastic islands in the BM. We consider the diagnosis of AAHS should be carefully made when macrophages phagocytosing blood cells are observed in BM smear without hyperferritinemia in elderly patients with autoimmune diseases.
authors.affiliation: Department of Internal Medicine, Hyogo Prefectural Tsukaguchi Hospital, 6-8-17 Minamitsukaguchi, Amagasaki, Hyogo 661-0012, Japan. rtabata@hp.pref.hyogo.jp
authors.forename: Rie
authors.initials: R
authors.lastname: Tabata
authors.forename: Chiharu
authors.initials: C
authors.lastname: Tabata
authors.forename: Makoto
authors.initials: M
authors.lastname: Terada
authors.forename: Tomoko
authors.initials: T
authors.lastname: Nagai
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D001172:Arthritis, Rheumatoid / Q000378:metabolism; D001323:Autoantibodies / Q000737:chemistry*; D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis / Q000276:immunology*; D001854:Bone Marrow Cells / Q000378:metabolism; D004900:Erythroblasts / Q000166:cytology*; D005260:Female; D005293:Ferritins / Q000097:blood; D050031:Hashimoto Disease / Q000378:metabolism; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000276:immunology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications / Q000175:diagnosis / Q000276:immunology*; D008297:Male; D012189:Retrospective Studies
chemical_list: D001323:Autoantibodies; D005293:Ferritins