Kidney non-tumor - Heavy chain deposition disease

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Kidney non-tumor

Associated with systemic conditions

Heavy chain deposition disease

Reviewers: Nikhil Sangle, M.D. (see Reviewers page)
Revised: 27 September 2012, last major update September 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Lymphoplasmacytic neoplasm that produces an abnormally truncated immunoglobulin heavy-chain protein that lacks associated light chains (eMedicine)
● Less common than light chain deposition disease
● May have milder hematological disorders than in light chain deposition disease (Clin Nephrol 2009;71:9)
● Associated with various lymphoproliferative disorders, but usually has features overlapping with cases previously reported as "vaguely nodular, polymorphous" lymphoplasmacytic lymphoma (Am J Surg Pathol 2012;36:534)

Case reports
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● 51 year old man with linear deposits of gamma 4 heavy chains along glomerular, tubular and vascular basement membrane and in mesangial regions (Mod Pathol 1994;7:874)
● 68 year old woman with gamma 1 heavy chain disease (Intern Med 2010;49:1411)
● Monotypic mu heavy chain mesangial deposits causing nodular glomerulosclerosis in woman without evident hematopoietic malignancy (Hum Pathol 2000;31:122)
● Follow up biopsy post-chemotherapy showed remarkable diminution of both nodular glomerular lesions and IgG heavy-chain deposits (Clin Nephrol 2008;69:383)

Micro images
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Various images

Immunofluorescence
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● IgG heavy chains along glomerular, tubular and vascular basement membranes and in mesangium
● No light chain deposition

Electron microscopy images
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Subendothelial electron-dense deposits and mesangial expansion

End of Kidney non-tumor > Associated with systemic conditions > Heavy chain deposition disease

Ref Updated: 7/27/12

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