A Case of Isolated Light Chain Deposition Disease in the Duodenum

Kim, Hee‐Jun; Park, Eunkyung; Lee, Tae Jin; Hyuk, Jae; Joο, Young Eun; Lee, Sang Jae

doi:10.3346/jkms.2012.27.2.207

Cited by 5 publications

(7 citation statements)

References 16 publications

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“…The clinical manifestations of aggregomas are related to the location of deposits. Rare cases of isolated aggregomas have also been reported in the brain, heart, pharynx and duodenum [1,8,9]. In 80 % of patients, the deposits are derived from kappa immunoglobulin light chain, as was the case with our patient.…”

Section: Discussionsupporting

confidence: 54%

Nonamyloid tumoral light-chain-deposition disease (aggregoma) of the paraspinal region

et al. 2015

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Aggregomas are rare localized masses of monoclonal nonamyloid immunoglobulin light-chain deposits. To date, there have been only a few reports of isolated aggregomas, with the majority detailing renal, lymph node and brain deposition. We present a rare case of paraspinal aggregoma in a 67-year-old female who presented with a complaint of cough and chest pain. Imaging demonstrated a left-sided paravertebral mass extending from T7-T10. Pathological analysis showed lamellar deposition of extracellular eosinophilic material with an associated lymphoplasmacytic nonamyloid infiltrate. To our knowledge, this is the first report of a paraspinal aggregoma. While exceedingly rare, this tumor can be included in the radiologic differential diagnosis of paravertebral soft tissue tumors in adults. The observation of our case adds to the limited understanding of the etiology, pathogenesis, natural history, and treatment of nonamyloid light-chain depositions.

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Section: Discussionsupporting

confidence: 54%

Nonamyloid tumoral light-chain-deposition disease (aggregoma) of the paraspinal region

et al. 2015

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“…As the clinical presentation in LCDD is known to depend on the number and nature of organs affected, deposition of different light chains does not seem to affect their clinical course [36]. The median duration of survival is approximately 4 years.…”

Section: Treatmentmentioning

confidence: 99%

Light chain deposition disease: novel biological insights and treatment advances

Jiménez-Zepeda

2012

Int J Lab Hematology

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Summary Light chain deposition disease (LCDD) is a monoclonal gammopathy characterized by nonamyloid deposition of immunoglobulin light chains in various organs. Most cases present with renal dysfunction, a ubiquitous feature of this disease, and in some instances, it may progress to end‐stage renal disease. Unfortunately, until now, no standard treatment has been established. The use of alkylating agents and steroids has been extensively reported. However, conventional chemotherapy response is generally limited with minor effects on kidney function. The use of novel agents such as bortezomib has shown a more rapid response with a dramatically important reduction of light chains in serum and/or urine in small series of cases. Furthermore, autologous stem cell transplantation has been reported as a feasible strategy in LCDD, able to prolong the dialysis‐free survival. Nonetheless, toxicity from these therapies should be considered carefully because most of patients might present with kidney dysfunction that could limit the use of some agents.

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“…[ 4 , 13 ] In addition, LC deposits in LCDD are not stained by Congo red and do not exhibit green birefringence under polarization. [ 5 , 9 ] Notably, diseases in which extracellular deposits show negative Congo red staining indicate Waldenstrom macroglobulinemia and amyloid-like fibronectin in addition to LCDD. [ 11 , 14 ] For differential diagnosis, clinical correlation, and mass spectrometric analysis may be helpful.…”

Section: Discussionmentioning

confidence: 99%

“…[ 6 , 20 ] However, lambda LC deposition was observed in 15 to 20% of cases, and LC and heavy chain depositions were observed in <10% of cases. [ 5 ] Our patient was a 55-year-old man who initially visited the hepatology clinic of our hospital presenting with symptomatic hepatic manifestations and was diagnosed with PCM based on further laboratory findings after assessing LCDD via liver biopsy. Although he lost 10 kg in 1 year, no other symptoms, particularly bone pain in the PCM, were reported before the appearance of dyspepsia, and jaundice was the chief complaint.…”

Section: Discussionmentioning

confidence: 99%

“…LCDD is a rare systemic disease characterized by tissue deposition of abnormal monoclonal LCs due to plasma cell dyscrasia. [3,4] As this disorder was first described in 1976 by Randall et al [3] in 2 patients with end-stage renal disease showing granular deposition of free LCs, [5] the kidneys are usually the most commonly affected organ and are responsible for early clinical manifestations, ranging from asymptomatic proteinuria to nephrotic syndrome or renal insufficiency. [4,[6][7][8] Notably, extrarenal involvement appears to be more common in patients with PCM.…”

Section: Discussionmentioning

confidence: 99%

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Plasma cell myeloma initially diagnosed as light-chain deposition disease on liver biopsy: A case report and literature review

et al. 2023

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Rationale: Light-chain deposition disease (LCDD) is a rare condition characterized by the abnormal deposition of monoclonal light chains (LCs) in multiple organs, leading to progressive organ dysfunction. Herein, we report a case of plasma cell myeloma initially diagnosed as LCDD on liver biopsy performed for prominent cholestatic hepatitis. Patient concerns: A 55-year-old Korean man complained of dyspepsia as the main symptom. On abdominal computed tomography performed at another hospital, the liver showed mildly decreased and heterogeneous attenuation with mild periportal edema. Preliminary liver function tests revealed abnormal results. The patient was treated for an unspecified liver disease; however, his jaundice gradually worsened, prompting him to visit our outpatient hepatology clinic for further evaluation. Magnetic resonance cholangiography revealed liver cirrhosis with severe hepatomegaly of unknown cause. A liver biopsy was performed for the diagnosis. Hematoxylin and eosin staining revealed diffuse extracellular amorphous deposits in perisinusoidal spaces with compressed hepatocytes. The deposits, which morphologically resembled amyloids, were not stained by Congo red but stained strongly positive for kappa LCs and weakly positive for lambda LCs. Diagnoses: Therefore, the patient was diagnosed with LCDD. Further systemic examination revealed a plasma cell myeloma. Interventions: Fluorescence in situ hybridization, cytogenetics, and next-generation sequencing tested in bone marrow showed no abnormalities. The patient initially received bortezomib/lenalidomide/dexamethasone as the treatment regimen for plasma cell myeloma. Outcomes: However, he died shortly thereafter because of coronavirus disease 2019 complications. Lessons: This case demonstrates that LCDD may present with sudden cholestatic hepatitis and hepatomegaly, and may be fatal if patients do not receive appropriate and timely treatment because of delayed diagnosis. Liver biopsy is useful for the diagnosis of patients with liver disease of unknown etiology.

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A Case of Isolated Light Chain Deposition Disease in the Duodenum

Cited by 5 publications

References 16 publications

Nonamyloid tumoral light-chain-deposition disease (aggregoma) of the paraspinal region

Nonamyloid tumoral light-chain-deposition disease (aggregoma) of the paraspinal region

Light chain deposition disease: novel biological insights and treatment advances

Plasma cell myeloma initially diagnosed as light-chain deposition disease on liver biopsy: A case report and literature review

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