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U06-11469

A 59-year-old Caucasian male presented with edema and was diagnosed with nephrotic syndrome in May 2006, having a serum albumin of 28 g/L. Initial work-up indicated proteinuria with a urine protein/creatinine ratio of 683–860 mg/mmol. Renal biopsy confirmed early-stage renal amyloidosis, with minimal amyloid deposition observed. Further investigations revealed cardiac amyloid involvement. The patient's past medical history included normal urinalysis in 2002 and 2003. The treatment plan includes autologous stem cell transplant scheduled for mid-September.

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U06-11469

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  1. U06-11469 #426820800 • Native (L) kidney • Proteinuria

  2. U06-11469 • 59 year old Caucasian male • no significant past medical history; normal u/a Nov 2002 and ?July 2003 (1+ protein on highly concentrated urine) • presented in December 2005 with new onset of peripheral edema; no renal work-up performed at that time • found to have nephrotic syndrome May 2006; serum albumin 28 g/L • work-up revealed serum creatinine of 88 µM with urine protein/creatinine ratio of 683 – 860 mg/mmol, negative hepatitis serology, no diabetes, normal complements, negative dsDNA, serum PEP=nephrotic syndrome, urine PEP=free lambda light chain • bone marrow Bx did not meet criteria for diagnosis of multiple myeloma • renal Bx results… • remainder of investigations….. • plan…..

  3. IF • No glomeruli are seen

  4. 10 nm mean diameter

  5. DiagnosisRenal Biopsy:Renal amyloidosis in an early stage with relatively small amounts of amyloid deposited and few changes by light microscopy.

  6. U06-11469 • 59 year old Caucasian male • no significant past medical history; normal u/a Nov 2002 and ?July 2003 (1+ protein on highly concentrated urine) • presented in December 2005 with new onset of peripheral edema; no renal work-up performed at that time • found to have nephrotic syndrome May 2006; serum albumin 28 g/L • work-up revealed serum creatinine of 88 µM with urine protein/creatinine ratio of 683 – 860 mg/mmol, negative hepatitis serology, no diabetes, normal complements, negative dsDNA, serum PEP=nephrotic syndrome, urine PEP=free lambda light chain • bone marrow Bx did not meet criteria for diagnosis of multiple myeloma • renal Bx revealed amyloidosis • remainder of investigations revealed cardiac amyloid with spickling and thickening of myocardium…..; normal function • plan autologous stem cell transplant mid-September

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