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

被引:8
作者
Stompor, Tomasz [1 ]
Perkowska-Ptasinska, Agnieszka [2 ]
Wojciechowska, Malgorzata [3 ]
Zajac, Katarzyna [1 ]
Chmielewska-Badziag, Agnieszka [1 ]
Pawlowska, Anna [1 ]
机构
[1] Univ Warmia & Mazury, Dept Nephrol Hypertens & Internal Med, PL-10561 Olsztyn, Poland
[2] Med Univ Warsaw, Dept Transplantol & Nephrol, Nephropathol Lab, Warsaw, Poland
[3] Olsztyn Reg Hosp, Dept Hematol, Olsztyn, Poland
关键词
Chronic kidney disease; crystalline nephropathy; fanconi syndrome; multiple myeloma; nephrotic syndrome; PLASMA-CELL DYSCRASIAS; PROXIMAL TUBULOPATHY; DEPOSITION; INCLUSIONS; DIAGNOSIS; SPECTRUM;
D O I
10.3109/0886022X.2014.884446
中图分类号
R5 [内科学]; R69 [泌尿科学(泌尿生殖系疾病)];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:795 / 799
页数:5
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