title: AH amyloidosis associated with an immunoglobulin heavy chain variable region (VH1) fragment: a case report.
abstract: We report a 67-year-old male patient who suffered from nephrotic syndrome and progressive renal dysfunction with monoclonal gammopathy (IgMkappa). Renal biopsy demonstrated amyloid deposition in glomeruli. Immunohistochemical studies of the renal amyloid using a number of antibodies, including anti-lambda and anti-kappa light chains, AA, beta(2)-microglobulin, and transthyretin, showed negative findings. Biochemical analysis of the deposited amyloid fibrils in gastroduodenal mucosa revealed that the amyloid fibrils were composed of an immunoglobulin heavy chain variable region (VH) fragment belonging to the VH1 subgroup, and a diagnosis of AH amyloidosis was made. In our institute, three patients with AH amyloidosis including the present one have been identified during the past 2 years, so AH amyloidosis seems to be by no means a rare disorder.
authors.affiliation: Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan.
authors.forename: Daigo
authors.initials: D
authors.lastname: Miyazaki
authors.forename: Masahide
authors.initials: M
authors.lastname: Yazaki
authors.forename: Takahisa
authors.initials: T
authors.lastname: Gono
authors.forename: Fuyuki
authors.initials: F
authors.lastname: Kametani
authors.forename: Ayako
authors.initials: A
authors.lastname: Tsuchiya
authors.forename: Masayuki
authors.initials: M
authors.lastname: Matsuda
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Takenaka
authors.forename: Yoshinobu
authors.initials: Y
authors.lastname: Hosh
authors.forename: Shu-ichi
authors.initials: S
authors.lastname: Ikeda
mesh_terms: D000368:Aged; D000595:Amino Acid Sequence; D000682:Amyloid / Q000235:genetics / Q000276:immunology / Q000378:metabolism; D000686:Amyloidosis / Q000150:complications / Q000175:diagnosis / Q000276:immunology* / Q000378:metabolism; D005753:Gastric Mucosa / Q000378:metabolism; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics / Q000378:metabolism*; D007135:Immunoglobulin Variable Region / Q000235:genetics / Q000378:metabolism*; D007413:Intestinal Mucosa / Q000378:metabolism; D007668:Kidney / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D008297:Male; D009404:Nephrotic Syndrome / Q000150:complications; D010265:Paraproteinemias / Q000150:complications
chemical_list: D000682:Amyloid; D007143:Immunoglobulin Heavy Chains; D007135:Immunoglobulin Variable Region