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

Journal of Pathology and Translational Medicine 2020년 54권 6호 p.504 ~ 507
김찬경, Brealey John, Jobert Anjelo, Nolan James,
소속 상세정보
김찬경 ( Kim Chan-Kyung ) - SA Pathology Department of Anatomical Pathology
 ( Brealey John ) - SA Pathology Department of Anatomical Pathology
 ( Jobert Anjelo ) - Royal Adelaide Hospital Central and Northern Adelaide Renal Transplantation Services
 ( Nolan James ) - SA Pathology Department of Anatomical Pathology

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.

키워드

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

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