ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferences
Light chain deposition disease
Print
Other Resources UpToDate PubMed

Light chain deposition disease

Print Images (1)
Contributors: Amirah Khan MD, Catherine Moore MD, Paritosh Prasad MD
Other Resources UpToDate PubMed

Synopsis

Light chain deposition disease (LCDD) is a rare disorder caused by deposition of non-amyloid kappa or lambda light chains in various parts of the body. Kappa light chains are involved 80% of the time. The kidneys are always affected, and the heart is affected 80% of the time. Light chain deposition can occur in other organs including the liver, spleen, gut, peripheral nerves, and brain.

The incidence of LCDD is unknown, with the median age of diagnosis being late in the fifth decade. 50%-60% of patients have associated multiple myeloma, and 17% have monoclonal gammopathy of unknown significance (MGUS) or no evidence of plasma cell proliferation. 30%-50% of cases are idiopathic.

Manifestations vary based on the affected organ system, although the kidneys are always involved. Signs and symptoms include proteinuria, hematuria, nephrotic syndrome, hypertension, heart failure, renal failure, arrhythmias, peripheral neuropathy, diarrhea, portal hypertension, liver failure, and dyspnea.

LCDD is pathologically similar to amyloid light chain (AL) amyloidosis, but 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. Definitive diagnosis is made with immunohistochemical staining of anti-kappa or anti-lambda light chain antibodies in affected tissue.

Treatment is geared toward controlling the plasma cell disorder and controlling organ dysfunction.

Codes

ICD10CM:
N08 – Glomerular disorders in diseases classified elsewhere

SNOMEDCT:
373604002 – Light-Chain Deposition Disease

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Differential on renal biopsy:

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Reviewed: 08/13/2019
Last Updated: 08/23/2019
Copyright © 2019 VisualDx®. All rights reserved.
Light chain deposition disease
Print 1 Images
Light chain deposition disease : Fatigue, Creatinine elevated, Hematuria, Lower extremity edema, Proteinuria, Vision loss, Dyspnea, Hypertension, BUN elevated
Copyright © 2019 VisualDx®. All rights reserved.