The morphological diagnosis of non-amyloid form of monoclonal immunoglobulin deposition disease in the kidney
Abstract
The papers shows the morphological findings of and clinical and demographic data on 61 patients with non-amyloid form of renal monoclonal immunoglobulin deposition disease (MIDD) unassociated with AL-amyloidosis and/or Bence Jones cast nephropathy: 40 cases of light-chain deposition disease, 18 cases of light-and-heavy chain deposition disease and 3 cases of heavy-chain deposition disease. According to the composition of the paraprotein deposits, the cases were distributed as follows: k (30), l (10), IgG/k (6), IgA/k (6), IgG/l (4), IgA/l (2) and g (3). Light microscopy revealed three variants of the glomerular pattern: diffuse nodular glomerulopathy (42.6%), diffuse mesangial dilation (27.9%) and intact glomeruli (29.5%). Varying severity of tubular atrophy was noted in 95% of cases. Tubular, glomerular, and smooth muscle basement membrane deposits substantially differed in immunofluorescent (100, 91.8 и 54.1%, respectively) and ultrastructural studies (55.7, 45.9 и 4.9%, respectively). Azotemia (68.9%) and proteinuria (55.8%) were most commonly revealed. Nephrotic syndrome and heavy proteinuria together made 27.9%.
About the Authors
O. A. Vorobyeva
Кафедра патологической анатомии с курсом цитологии, Санкт-Петербургская медицинская академия последипломного образования, г. Санкт Петербург, Россия
Russian Federation
Russian Federation
C. . Nast
Отдел патологии и лабораторной медицины, Медицинский центр Cedars-Sinai, Лос-Анджелес, США
Russian Federation
Russian Federation
A. . Cohen
Отдел патологии и лабораторной медицины, Медицинский центр Cedars-Sinai, Лос-Анджелес, США
Russian Federation
Russian Federation
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Review
For citations:
Vorobyeva O.A., Nast C., Cohen A. The morphological diagnosis of non-amyloid form of monoclonal immunoglobulin deposition disease in the kidney. Nephrology and Dialysis. 2007;9(4):421-426. (In Russ.)
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