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Diagnosis: Light chain deposition disease

Light-chain deposition disease (LCDD) is the deposition of monoclonal, amorphous, noncongophilic light chains in multiple organs. In LCDD there is an infiltration of light chains involving multiple organs. Renal involvement is a constant feature which include renal insufficiency, proteinuria, and nephrotic syndrome. Extrarenal involvement is primarily noted at autopsy and is usually confined to the perivascular regions of the affected organs. Approximately 50-60% of patients with LCDD have associated lymphoproliferative disorder, most commonly multiple myeloma. The remaining cases develop LCDD in the setting of progression of monoclonal gammopathy of unknown significance (MGUS) or with no evidence of neoplastic plasma cell proliferation. Approximately 85% of cases are associated with kappa light-chain deposition. A monoclonal protein of the same light-chain type is usually demonstrated in serum or urine, but approximately 25% of patients have no demonstrable light chain in serum or urine by immunoelectrophoresis or immunofixation. Even in the absence of a monoclonal light chain in serum or urine, immunofluorescence usually demonstrates a monoclonal population of plasma cells in the bone marrow of these patients. The renal lesion is usually a nodular mesangial lesion that is often indistinguishable from diabetic lesions by light microscopy. Immunofluorescence or electron microscopy are essential in making the diagnosis, and the findings on renal biopsy are often the first evidence of LCDD. Treatment with high-dose chemotherapy with or without autologous stem cell transplantation may result in disease stabilization and/or improvement in end-organ damage (Jayamohan Y, Sacher RA, Fanning SR. Light-Chain Deposition Disease. In eMedicine. [Link])

In our patient, a bone marrow biopsy, several months after renal biopsy, demonstrated multiple myeloma. In the follow-up he presented polyneuropathy and several episodes of supraventricular tachycardia. He is on chemotheraphy treatment.

The renal features on renal biopsy and immunofluorescence are very characteristic of the disease.

See the chapter Amyloidosis, multiple myeloma, light chain deposition disease... of our Tutorial.

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Bibliography

• Stratta P, Gravellone L, Cena T, Rossi D, Gaidano G, Fenoglio R, Lazzarich E, Quaglia M, Airoldi A, Bozzola C, Monga G, Valente G, Canavese C, Magnani C. Renal outcome and monoclonal immunoglobulin deposition disease in 289 old patients with blood cell dyscrasias: A single center experience. Crit Rev Oncol Hematol. 2010 May 28. [PubMed link]
• Jayamohan Y, Sacher RA, Fanning SR. Light-Chain Deposition Disease. In eMedicine. [http://emedicine.medscape.com/article/202585-overview]
• Martín Herrera C, Suñer Poblet M, Cabrera R, Díaz Pedrero M, Fernández Alonso J. Light chain deposition disease. Experience in our environment. Nefrologia. 2008;28(5):539-42. [PubMed link] [Free full text]
• Gokden N, Barlogie B, Liapis H. Morphologic heterogeneity of renal light-chain deposition disease. Ultrastruct Pathol. 2008 Jan-Feb;32(1):17-24. [PubMed link]
• Ferrario F, Rastaldi MP. Histopathological atlas of renal diseases: light chain deposition disease. J Nephrol. 2005 Sep-Oct;18(5):499-502. [PubMed link]
• Pozzi C, D'Amico M, Fogazzi GB, Curioni S, Ferrario F, Pasquali S, Quattrocchio G, Rollino C, Segagni S, Locatelli F. Light chain deposition disease with renal involvement: clinical characteristics and prognostic factors. Am J Kidney Dis. 2003 Dec;42(6):1154-63. [PubMed link]

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