AH amyloidosis associated with an immunoglobulin heavy chain variable region (VH1) fragment: a case report.
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.
Miyazaki
Daigo
D
Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan.
Yazaki
Masahide
M
Gono
Takahisa
T
Kametani
Fuyuki
F
Tsuchiya
Ayako
A
Matsuda
Masayuki
M
Takenaka
Yoshiaki
Y
Hosh
Yoshinobu
Y
Ikeda
Shu-ichi
S
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
D000682:Amyloid; D007143:Immunoglobulin Heavy Chains; D007135:Immunoglobulin Variable Region