J Korean Med Sci.  2012 Feb;27(2):207-210. 10.3346/jkms.2012.27.2.207.

A Case of Isolated Light Chain Deposition Disease in the Duodenum

• Kim HJ
• Park E
• Lee TJ
• Do JH
• Cha YJ
• Lee SJ

• KMID: 1120160
• DOI: http://doi.org/10.3346/jkms.2012.27.2.207

Abstract

Light chain deposition disease (LCDD) is a rare disorder associated with a clonal proliferation of plasma cells, which synthesize abnormal monoclonal immunoglobulin light chains. LCDD is characterized by systemic deposition of light chains in various organs, with the kidneys being most commonly affected. There have been few reports of isolated LCDD. We report a rare case of LCDD limited to a duodenal polyp. A 63-yr-old man visited our hospital for health screening without symptoms in 2009. On gastrofiberscopy, a duodenal polyp was observed. The biopsy showed diffuse infiltration by atypical plasma cells, which were positive for kappa-type light chains by immunohistochemistry. While the patient refused further management, we could find no evidence of recurrence until 2 yr after the initial diagnosis. It has been reported that isolated LCDD has relatively good prognosis compared to systemic LCDD. However, treatment for this disease has not been established yet.

Keyword

Light Chain Deposition Disease (LCDD); Duodenal Polyp; Isolated LCDD

MeSH Terms

Duodenum/pathology
Endoscopy, Gastrointestinal
Humans
Immunoglobulin kappa-Chains/*immunology/metabolism
Immunohistochemistry
Intestinal Mucosa/*pathology
Male
Middle Aged
Paraproteinemias/*diagnosis/pathology
Tomography, X-Ray Computed

Figure

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  Fig. 1 Esophagogastroduodenoscopy showing polypoid mass in the duodenal bulb.

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  Fig. 2 Histopathology of duodenal mucosa. (A) Deposition of pale pink amorphous materials and infiltration of plasma cells in the lamina propria (H&E stain, × 400). (B) Negative reaction on Congo red staining (× 400). (C) Positive reaction in plasma cells with anti-κ light chain antibodies (IHC stain, × 400). (D) Negative reaction with anti-λ light chain antibodies (IHC stain, × 400).

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  Fig. 3 Abdominal CT showing multiple polypoid mass in the duodenum. There are multiple inhomogeneous polypoid masses in the duodenum (arrowheads).

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