What is the pathophysiology of extrarenal light-chain deposition disease (LCDD)?

Updated: Sep 26, 2019
  • Author: Swapna Boppana, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Symptomatic extrarenal LCDD is rare. [19] The liver is the most frequent site involved. [10, 20] The deposits in the liver are usually confined to the sinusoids and basement membrane of biliary ducts, without associated parenchymal lesions. The degree of liver involvement does not correlate with the amount of light chains deposited. [20] Patients may develop cirrhosis and/or portal hypertension and may die from liver failure. [10]

The cardiac manifestations include restrictive cardiomyopathy, cardiomegaly, congestive heart failure, and arrhythmias. [21, 22, 23] Echocardiography and cardiac catheterization may reveal diastolic dysfunction and decreased myocardial compliance. [24]

Pulmonary LCDD is rare, and it usually damages the lung parenchyma. Involvement of the large airways has recently been reported. [25] Nodular and diffuse pulmonary interstitial diseases of the lungs have been described. [26, 27, 28]

LCDD can affect peripheral nerves, resulting in polyneuropathy. [29] Isolated LCDD restricted to the brain has been reported as well, wherein the periventricular foci of intracerebral vessels were overloaded with amorphous, eosinophilic material that stained for lambda light chains. [30] In general, the blood-brain barrier protects the central nervous system (CNS) from circulating, misfolded proteins, but cases of intracerebral amyloidoma have been reported. [31]

LCDD can also affect other sites, such as lymph nodes, skin, spleen, pancreas, bone marrow, thyroid gland, adrenal gland, and abdominal blood vessels. [9]

LCDD is associated with multiple myeloma is about 58% of cases. [5] LCDD could be present at diagnosis of a new plasma cell dyscrasia or could represent an extramedullary manifestation of multiple myeloma while relapsing after chemotherapy. [24] LCDD can also complicate other B-cell lymphomas, such as lymphoplasmacytic lymphoma, marginal-zone lymphoma, and chronic lymphocytic lymphoma. [32]


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