J Pathol Transl Med.  2020 Nov;54(6):504-507. 10.4132/jptm.2020.09.18.

A case of monoclonal gammopathy of renal significance presenting as atypical amyloidosis with IgA lambda paraproteinemia

• Kim C
• Brealey J
• Jobert A
• Nolan J

• KMID: 2509504
• DOI: http://doi.org/10.4132/jptm.2020.09.18

Abstract

Monoclonal gammopathy of renal significance is defined as any B cell or plasma cell clonal lymphoproliferation which neither causes tumor complications nor meets any current hematological criteria for specific therapy, with one or more kidney lesions related to the produced monoclonal immunoglobulin, such as amyloidosis. A 50-year-old male presented with heavy proteinuria and blood tests showing IgA and Lambda paraproteinemia. Light microscopy showed mesangial eosinophilic ground substance extending into the capillary loops, and positive staining within the glomeruli and vessel walls for amyloid P immunohistochemistry was also noted. Immunofluorescence showed positive staining for IgA and Lambda in the mesangia and capillary loops. Electron microscopy exhibited organized fibrils measuring 4–5 nm in diameter in the mesangia, glomerular basement membranes and vessel walls. We interpreted the overall findings as atypical renal amyloidosis with IgA and Lambda deposition on immunofluorescence. Further amyloid typing using laser microdissection-liquid chromatography and mass spectrometry will be useful.

Keyword

Monoclonal gammopathy of renal significance; AHL amyloidosis; Immunoglobulin A; Atypical amyloidosis

Figure

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  Fig. 1. Hematoxylin and eosin stain and Amyloid P immunohistochemistry. (A) The glomerulus shows mesangial eosinophilic ground substance which extends into the capillary loops. (B) Positive staining is seen within the glomerulus with amyloid P immunohistochemistry.

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  Fig. 2. Immunofluorescence and electron microscopy. (A) Positive mesangial and capillary loop staining with IgA on immunofluorescence. (B) Positive mesangial and capillary loops staining with Lambda on immunofluorescence. (C) The fine fibrillary material (F) is present adjacent to the mesangial cell (M) (×40,000). The inset shows amyloid-like fibrils measuring approximately 5 nm in diameter (×120,000).

Reference

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