Literature DB >> 24512271

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

Tomasz Stompór1, Agnieszka Perkowska-Ptasińska, Małgorzata Wojciechowska, Katarzyna Zając, Agnieszka Chmielewska-Badziąg, Anna Pawłowska.   

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.

Entities:  

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

Mesh:

Year:  2014        PMID: 24512271     DOI: 10.3109/0886022X.2014.884446

Source DB:  PubMed          Journal:  Ren Fail        ISSN: 0886-022X            Impact factor:   2.606


  1 in total

1.  Crystalloid podocytopathy with focal segmental glomerulosclerosis in PCM: a case report.

Authors:  You La Jeon; Woo In Lee; Yujin Choi; So Young Kang; Myeong Hee Kim; Sung-Jig Lim; Sang Ho Lee
Journal:  Diagn Pathol       Date:  2015-12-16       Impact factor: 2.644

  1 in total

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