Unusual manifestation of crystalline light chain tubulopathy in patient with multiple myeloma: case report and review of the literature

Ren Fail. 2014 Jun;36(5):795-9. doi: 10.3109/0886022X.2014.884446. Epub 2014 Feb 10.

Abstract

Multiple myeloma (MM) is the second most common hematological malignancy, with an annual incidence in Europe and the USA of about 4-6 cases per 100,000. Several forms of renal disease are found in the course of MM, including: cast nephropathy, light chain (LC) deposition disease and primary amyloidosis. Less frequent forms include: acute and chronic tubulopathies, neoplastic plasma cell infiltration and interstitial nephritis. In this paper, we discuss a case of 53-year-old male patient with MM who presented with massive proteinuria (24 g/24 h), mild renal insufficiency (eGFR 43 mL/min), and Fanconi-like syndrome (as reflected by normoglycemic glycosuria). In kidney biopsy glomeruli were normal, whereas abundant AFOG-positive deposits were found in the cytoplasm of proximal tubular epithelial cells. These deposits were strongly positive for kappa light chains on immunofluorescence. Electron microscopy revealed electron-dense, intracytoplasmic crystalloid deposits of variable shape (needle-shaped, round and rectangular), and size in the proximal tubular cells. This unusual variant of microscopic renal lesions in the course of MM coupled with coincidence of Fanconi-like and nephrotic syndrome as a clinical manifestation has not been reported to date.

Keywords: Chronic kidney disease; crystalline nephropathy; fanconi syndrome; multiple myeloma; nephrotic syndrome.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Fanconi Syndrome / complications*
  • Fanconi Syndrome / pathology
  • Humans
  • Kidney / ultrastructure
  • Male
  • Middle Aged
  • Multiple Myeloma / complications*
  • Multiple Myeloma / diagnosis
  • Proteinuria / etiology*
  • Proteinuria / pathology
  • Renal Insufficiency / complications*
  • Renal Insufficiency / pathology