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Light chain deposition disease
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Light chain deposition disease

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Contributors: Catherine Moore MD
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Synopsis

A disorder where plasma cells secrete immunoglobulin light chains of only one type, kappa or lambda (usually kappa). These immunoglobulins deposit in tissues, typically kidneys, heart, and/or liver, and lead to organ dysfunction. Renal deposition may be glomerular or in the tubular basement membranes. Clinical presentation varies by site of immunoglobulin deposition. Patients with glomerular deposition will have disruption of the filtration barrier and present with significant albuminuria and nephrotic syndrome. Those with tubular deposition may present with renal insufficiency and minimal proteinuria.

The proteinuria evident in light-chain deposition disease (LCDD) is mainly albumin, in contrast to myeloma cast nephropathy (with significant urinary light chain excretion).

LCDD is pathologically similar to amyloid light-chain (AL) amyloidosis, but the pathologic immunoglobulins do not form amyloid fibrils and do not stain Congo red. Serum and urine protein electrophoresis may not detect a monoclonal protein, but serum-free light chain analysis has increased sensitivity.

LCDD is most commonly associated with multiple myeloma but is also seen in patients with lymphoma or Waldenstrom macroglobulinemia. 30%-50% of cases are idiopathic. Treatment is geared towards controlling the plasma cell disorder.

Codes

ICD10CM:
N08 – Glomerular disorders in diseases classified elsewhere

SNOMEDCT:
373604002 – Light-Chain Deposition Disease

Differential Diagnosis & Pitfalls

Differential on renal biopsy:

Best Tests

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References

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Last Updated: 06/28/2016
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Light chain deposition disease
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Light chain deposition disease : Fatigue, Creatinine elevated, Hematuria, Lower extremity edema, Proteinuria, Dyspnea, BUN elevated, BP increased
Copyright © 2019 VisualDx®. All rights reserved.