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

American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation
Takahisa GonoShu-ichi Ikeda

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.

References

Sep 1, 1990·Proceedings of the National Academy of Sciences of the United States of America·M EulitzA Solomon
Feb 1, 1994·American Journal of Hematology·A SolomonC Murphy
May 20, 1998·Biochemical and Biophysical Research Communications·K E OlsenP Westermark
Apr 28, 2004·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·Masahide YazakiShu-ichi Ikeda
Oct 14, 2004·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·Takahisa GonoShu-ichi Ikeda
Jul 16, 2005·Virchows Archiv : an International Journal of Pathology·Batia KaplanMordechai R Kramer

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Citations

Feb 16, 2010·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·Jill VanmassenhoveFadi Fakhouri
Jul 18, 2008·Clinical Journal of the American Society of Nephrology : CJASN·Vincent AudardPierre Ronco
Jul 16, 2008·Cardiovascular Pathology : the Official Journal of the Society for Cardiovascular Pathology·A Bernard CollinsJames R Stone
Apr 11, 2007·Current Opinion in Nephrology and Hypertension·Maria M Picken
Jul 11, 2009·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·Nagaaki KatohShu-Ichi Ikeda
May 20, 2008·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·Daigo MiyazakiShu-ichi Ikeda

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