Open AccessKappa Light Chain Nodular Glomerulosclerosis with Conspicuous Crescent Formation and Tubulointerstitial Injury
Author(s) -
Nakamoto Yasushi,
Kawamura Kohichi,
Mamiya Shigeo,
Yasuda Tadashi,
Imai Hirokazu,
Miura Akira B.,
Hayashi Masato
Publication year - 1992
Publication title -
acta patholigica japonica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
ISSN - 0001-6632
DOI - 10.1111/j.1440-1827.1992.tb03250.x
Subject(s) - medicine , pathology , renal biopsy , plasma cell dyscrasia , multiple myeloma , immunoglobulin light chain , glomerulosclerosis , differential diagnosis , glomerulonephritis , biopsy , proteinuria , bone marrow , kidney , antibody , immunology
We describe a 39‐year‐old man who developed kappa light chain nodular glomerulosclerosis with superimposed conspicuous crescent formation and extensive tubulointerstitial injury. The clinical picture was characterized by nephrotic syndrome and rapidly progressive glomerulonephritis. Incessantly progressive loss of renal function culminated in irreversible renal failure 7 weeks after initial manifestation of renal insufficiency. The patient has since been maintained on thrice weekly hemodialysis with chemotherapy for five years. At the time of pathologic diagnosis by renal biopsy, there was no evidence of multiple myeloma, and no serum M‐component or Bence‐Jones proteinuria was detected. An initial bone marrow aspirate revealed the presence of 0.6% atypical lymphocytes as the sole abnormality, although these were later identified as atypical plasma cells. These cells had also infiltrated the renal interstitium. Crescentic kappa light chain nodular glomerulosclerosis lacking evidence of plasma cell dyscrasia should be included in the differential diagnosis of rapidly progressive glomerulonephritis.