A case of primary (AL) amyloidosis with predominantly vascular amyloid deposition in the kidney

CEN Case Reports
Yoichi MurakamiHideki Matsushima

Abstract

We report a 70-year-old man with primary (AL) amyloidosis with predominantly vascular deposition of amyloid diagnosed by renal biopsy, who was successfully treated using two chemotherapy regimens. There was rapid elevation of the serum creatinine level without remarkable proteinuria or hematuria. Renal histological examination showed some thickened arterial walls with amyloid fibril accumulation, and only a small amount of amyloid deposition in the glomeruli. Immunohistochemical examination was positive for anti-kappa staining. Serum immunoelectrophoresis and immunofixation testing did not show monoclonal proteins, and urine immunoelectrophoresis did not show Bence-Jones proteins. Serum free light chain (FLC) analysis showed that the serum FLC level and FLC kappa/lambda ratio were abnormally high for his renal function. He received two courses of VAD (vincristine, doxorubicin, and dexamethasone), followed by BD (bortezomib and dexamethasone), resulting in a hematologic partial response. Renal amyloidosis with vascular-limited amyloid deposition has few urinary findings. Early diagnosis of this condition is challenging, because kidney biopsies are not usually performed in patients without significant urinary findings. We suggest...Continue Reading

References

Mar 1, 1987·The American Journal of Medicine·M A DustonA S Cohen
Feb 1, 1995·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·B I FreedmanR G Appel
Jun 10, 2000·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·W C O'Neill
May 23, 2006·Acta histochemica·Per WestermarkStina Enqvist
Nov 10, 2006·Journal of the American Society of Nephrology : JASN·Laura M Dember
Feb 22, 2008·Haematologica·Tilmann BochtlerStefan O Schonland
Oct 24, 2008·Clinical Journal of the American Society of Nephrology : CJASN·Colin A HutchisonPaul Cockwell
Jan 21, 2010·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·Efstathios KastritisGiovanni Palladini
Nov 10, 2011·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·Alfonso EirinNelson Leung
Nov 2, 2012·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·Jean D SipeUNKNOWN Nomenclature Committee of the International Society of Amyloidosis
Nov 28, 2012·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·Ying YaoWan-Zhong Zou

❮ Previous
Next ❯

Citations

Oct 31, 2020·Frontiers in Immunology·Mikhail InyushinLilia Kucheryavykh

❮ Previous
Next ❯

Related Concepts

Related Feeds

Bipolar Disorder

Bipolar disorder is characterized by manic and/or depressive episodes and associated with uncommon shifts in mood, activity levels, and energy. Discover the latest research this illness here.