Systemic and rapidly progressive light-chain deposition disease initially presenting as tubulointerstitial nephritis

CEN Case Reports
Satoko TakahashiHiroshi Sato

Abstract

A 42-year-old woman was admitted to a hospital after first-time detection of proteinuria and hematuria during a routine medical check-up. Because her serum creatinine level had rapidly increased from 0.9 to 3.2 mg/dl since measurement 3 months earlier, she was referred to our hospital. Renal biopsy revealed extensive tubular atrophy and interstitial fibrosis with mild leukocyte infiltration. Glomeruli showed minimal changes, and no immunoglobulin or complement deposition was observed by immunofluorescence. Oral prednisolone was commenced under the diagnosis of chronic tubulointerstitial nephritis, and she discharged once. However, its effects were transient; her renal function deteriorated rapidly and hemodialysis was initiated 5 months after her initial check-up. On readmission, urinary Bence-Jones protein κ-type was detected, and examination of bone marrow led to a diagnosis of Bence-Jones κ-type multiple myeloma. Light-chain staining using a renal biopsy specimen obtained 2 months earlier showed κ-light-chain deposition on tubular basement membranes but not glomeruli. Despite undergoing chemotherapy with vincristine, doxirubicin, and dexamethasone, the patient died suddenly from a cardiac arrhythmia. Autopsy showed κ-light-c...Continue Reading

References

Feb 1, 1976·The American Journal of Medicine·R E RandallW J Still
Jul 1, 1992·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·R L HeilmanR A Kyle
Jan 1, 1988·American Journal of Nephrology·V S VenkataseshanM H Goldstein
Jul 1, 1984·Kidney International·D GanevalJ P Grünfeld
Nov 1, 1980·The American Journal of Medicine·J L Preud'hommeM Seligmann
Jan 14, 2004·Kidney International·Bruno RoyerJean-Paul Fermand
Aug 21, 2007·Clinical Journal of the American Society of Nephrology : CJASN·Pierre RoncoPierre Aucouturier

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