title: Autoimmune pancreatitis associated with myelodysplastic syndrome.
abstract: A 65-year-old man with myelodysplastic syndrome (MDS) was admitted for progressive jaundice. Diffuse pancreatic swelling and stricture of the main pancreatic duct were observed with elevated serum levels of direct bilirubin, aspartate transaminase, alanine transaminase, alkaline phosphatase, gammaGTP and amylase, and impaired glucose tolerance. Serum IgG and IgG4 levels were highly elevated, and both the direct antiglobulin test and platelet-associated IgG were positive. He was diagnosed with autoimmune pancreatitis associated with MDS, and biliary drainage followed by immunosuppressive therapy ameliorated the jaundice and laboratory findings. In addition to diffuse pancreatic FDG accumulation, fine incorporations of FDG to the lachrymal and submandibular glands were demonstrated, suggesting the recently proposed IgG4+ multiorgan lymphoproliferative syndrome (MOLPS). The etiology of IgG4+ MOLPS is still unknown; however, autoantibodies to blood cells in this case suggested that the autoimmune mechanism, which is caused by abnormal immune functions in MDS patients, might be involved in the pathogenesis of IgG4+ MOLPS.
authors.affiliation: Department of Internal Medicine, Hyogo Prefectural Tsukaguchi Hospital, Hyogo, 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: Tomoko
authors.initials: T
authors.lastname: Okamoto
authors.forename: Konosuke
authors.initials: K
authors.lastname: Omori
authors.forename: Makoto
authors.initials: M
authors.lastname: Terada
authors.forename: Tomoko
authors.initials: T
authors.lastname: Nagai
mesh_terms: D000368:Aged; D000681:Amylases / Q000097:blood; D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D001379:Azathioprine / Q000627:therapeutic use; D001652:Bile Ducts / Q000473:pathology; D001663:Bilirubin / Q000097:blood; D001773:Blood Cells / Q000276:immunology / Q000473:pathology; D001853:Bone Marrow / Q000473:pathology; D006801:Humans; D008297:Male; D009190:Myelodysplastic Syndromes / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D010179:Pancreas / Q000473:pathology; D010183:Pancreatic Ducts / Q000473:pathology; D010195:Pancreatitis / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D010198:Pancytopenia / Q000188:drug therapy / Q000473:pathology; D010976:Platelet Count; D011239:Prednisolone / Q000627:therapeutic use
chemical_list: D001323:Autoantibodies; D011239:Prednisolone; D000681:Amylases; D001379:Azathioprine; D001663:Bilirubin