AH amyloidosis associated with lymphoplasmacytic lymphoma secreting a monoclonal gamma heavy chain carrying an unusual truncated D segment.

Abstract

To date, the presence of amyloidosis associated with immunoglobulin heavy chain (AH amyloidosis) was reported in only 7 cases. Although AH amyloidosis is caused mainly by plasma cell dyscrasia, as in AL amyloidosis, we report a 61-year-old patient who presented with nephrotic syndrome caused by AH amyloidosis associated with lymphoplasmacytic lymphoma. Biochemical and molecular analyses of the deposited amyloid fibrils and heavy-chain genes of lymphocytes showed that proliferative lymphoma cells produced a gamma heavy chain, not a mu heavy chain, which carried an unusual truncated diversity (D) segment of the variable region. Our results indicate that production of the abnormal heavy chain caused by the partially deleted D segment gene is responsible for gamma heavy-chain-related amyloid fibril formation in this patient.

• Authors: Takahisa Gono, Masahide Yazaki, Tomohisa Fushimi, Takefumi Suzuki, Tsuyoshi Uehara, Kenji Sano, Fuyuki Kametani, Nobuo Ito, Masahiro Matsushita, Shigeo Nakamura, Yoshinobu Hoshii, Masayuki Matsuda, Shu-ichi Ikeda
• Journal: American journal of kidney diseases : the official journal of the National Kidney Foundation (Am J Kidney Dis) Vol. 47 Issue 5 Pg. 908-14 (May 2006) ISSN: 1523-6838 [Electronic] United States
• PMID: 16632032 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Immunoglobulin Heavy Chains
• Immunoglobulin gamma-Chains

Topics

• Amyloidosis (complications, immunology)
• Female
• Humans
• Immunoglobulin Heavy Chains
• Immunoglobulin gamma-Chains (analysis, genetics, immunology)
• Leukemia, Lymphocytic, Chronic, B-Cell (complications, immunology)
• Middle Aged