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Light chain deposition disease

MedGen UID:
65953
•Concept ID:
C0238239
•
Neoplastic Process
Synonym: Light chain disease
SNOMED CT: Light chain deposition disease (373604002); Light chain disease (51319002)
 
Monarch Initiative: MONDO:0019730
Orphanet: ORPHA93558

Definition

A rare non-amyloid monoclonal immunoglobulin deposition disease characterized by deposition of abnormal immunoglobulin light chains in the kidneys, resulting in nephrotic syndrome and renal failure. Symptomatic extrarenal deposition is uncommon, although hepatic, cardiac, and neural deposits have been reported. The condition frequently occurs in association with multiple myeloma or in patients with M protein and marrow plasma cells at monoclonal gammopathy of undetermined significance levels. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLight chain deposition disease

Professional guidelines

PubMed

Diagnosis of cystic lung diseases: a position statement from the UK Cystic Lung Disease Rare Disease Collaborative Network.
Johnson SR, Shaw DE, Avoseh M, Soomro I, Pointon KS, Kokosi M, Nicholson AG, Desai SR, George PM
Thorax 2024 Mar 15;79(4):366-377. doi: 10.1136/thorax-2022-219738. PMID: 38182428
The pathogenesis of renal injury and treatment in light chain deposition disease.
Wang Q, Jiang F, Xu G
J Transl Med 2019 Nov 25;17(1):387. doi: 10.1186/s12967-019-02147-4. PMID: 31767034Free PMC Article
Light chain deposition disease: novel biological insights and treatment advances.
Jimenez-Zepeda VH
Int J Lab Hematol 2012 Aug;34(4):347-55. Epub 2012 Apr 4 doi: 10.1111/j.1751-553X.2012.01419.x. PMID: 22471811

Recent clinical studies

Etiology

Paraprotein-Mediated Glomerular Diseases.
Miao J, Herrmann SM, Obaidi Z, Caza T, Bonilla M
Adv Kidney Dis Health 2024 Jul;31(4):358-373. doi: 10.1053/j.akdh.2024.02.005. PMID: 39084761
Ultra-rare cystic disease.
Elia D, Torre O, Cassandro R, Caminati A, Harari S
Eur Respir Rev 2020 Sep 30;29(157) Epub 2020 Sep 2 doi: 10.1183/16000617.0163-2019. PMID: 32878971Free PMC Article
Pulmonary nodular and cystic light chain deposition disease: A retrospective review of 10 cases.
Baqir M, Moua T, White D, Yi ES, Ryu JH
Respir Med 2020 Apr;164:105896. Epub 2020 Feb 14 doi: 10.1016/j.rmed.2020.105896. PMID: 32217287
Diffuse Cystic Lung Diseases: Diagnostic Considerations.
Xu KF, Feng R, Cui H, Tian X, Wang H, Zhao J, Huang H, Zhang W, Lo BH
Semin Respir Crit Care Med 2016 Jun;37(3):457-67. Epub 2016 May 27 doi: 10.1055/s-0036-1580690. PMID: 27231867
Diffuse Cystic Lung Disease. Part II.
Gupta N, Vassallo R, Wikenheiser-Brokamp KA, McCormack FX
Am J Respir Crit Care Med 2015 Jul 1;192(1):17-29. doi: 10.1164/rccm.201411-2096CI. PMID: 25906201Free PMC Article

Diagnosis

Paraprotein-Mediated Glomerular Diseases.
Miao J, Herrmann SM, Obaidi Z, Caza T, Bonilla M
Adv Kidney Dis Health 2024 Jul;31(4):358-373. doi: 10.1053/j.akdh.2024.02.005. PMID: 39084761
Diagnosis of cystic lung diseases: a position statement from the UK Cystic Lung Disease Rare Disease Collaborative Network.
Johnson SR, Shaw DE, Avoseh M, Soomro I, Pointon KS, Kokosi M, Nicholson AG, Desai SR, George PM
Thorax 2024 Mar 15;79(4):366-377. doi: 10.1136/thorax-2022-219738. PMID: 38182428
Lymphangioleiomyomatosis and Other Cystic Lung Diseases.
Koslow M, Lynch DA, Cool CD, Groshong SD, Downey GP
Immunol Allergy Clin North Am 2023 May;43(2):359-377. doi: 10.1016/j.iac.2023.01.003. PMID: 37055093Free PMC Article
Ultra-rare cystic disease.
Elia D, Torre O, Cassandro R, Caminati A, Harari S
Eur Respir Rev 2020 Sep 30;29(157) Epub 2020 Sep 2 doi: 10.1183/16000617.0163-2019. PMID: 32878971Free PMC Article
Cystic Lung Diseases: Algorithmic Approach.
Raoof S, Bondalapati P, Vydyula R, Ryu JH, Gupta N, Raoof S, Galvin J, Rosen MJ, Lynch D, Travis W, Mehta S, Lazzaro R, Naidich D
Chest 2016 Oct;150(4):945-965. Epub 2016 May 13 doi: 10.1016/j.chest.2016.04.026. PMID: 27180915Free PMC Article

Therapy

Treatment of idiopathic light chain deposition disease: complete remission with bortezomib and dexamethasone.
Souto Filho JT, Monteiro JM, Andrade IB
J Bras Nefrol 2016 Dec;38(4):450-454. doi: 10.5935/0101-2800.20160071. PMID: 28001187
Light Chain Deposition Disease After Kidney Transplantation With Long Graft Survival: Case Report.
Kuppachi S, Holanda D, Thomas CP
Transplant Proc 2016 Jan-Feb;48(1):255-8. doi: 10.1016/j.transproceed.2015.12.009. PMID: 26915878
Cystic and nodular lung disease.
Richards JC, Lynch DA, Chung JH
Clin Chest Med 2015 Jun;36(2):299-312, ix. Epub 2015 Apr 2 doi: 10.1016/j.ccm.2015.02.011. PMID: 26024606
Light chain deposition disease: novel biological insights and treatment advances.
Jimenez-Zepeda VH
Int J Lab Hematol 2012 Aug;34(4):347-55. Epub 2012 Apr 4 doi: 10.1111/j.1751-553X.2012.01419.x. PMID: 22471811
Light chain deposition disease with renal involvement: clinical characteristics and prognostic factors.
Pozzi C, D'Amico M, Fogazzi GB, Curioni S, Ferrario F, Pasquali S, Quattrocchio G, Rollino C, Segagni S, Locatelli F
Am J Kidney Dis 2003 Dec;42(6):1154-63. doi: 10.1053/j.ajkd.2003.08.040. PMID: 14655186

Prognosis

Diagnosis of cystic lung diseases: a position statement from the UK Cystic Lung Disease Rare Disease Collaborative Network.
Johnson SR, Shaw DE, Avoseh M, Soomro I, Pointon KS, Kokosi M, Nicholson AG, Desai SR, George PM
Thorax 2024 Mar 15;79(4):366-377. doi: 10.1136/thorax-2022-219738. PMID: 38182428
Coexistence of amyloidosis and light chain deposition disease in the heart.
Sobue Y, Takemura G, Kawamura S, Yano T, Kanamori H, Morimoto SI, Matsuo H
Cardiovasc Pathol 2021 Mar-Apr;51:107315. Epub 2020 Nov 29 doi: 10.1016/j.carpath.2020.107315. PMID: 33264681
Diffuse cystic lung diseases.
Ryu JH, Tian X, Baqir M, Xu K
Front Med 2013 Sep;7(3):316-27. Epub 2013 May 11 doi: 10.1007/s11684-013-0269-z. PMID: 23666611
Light chain deposition disease with renal involvement: clinical characteristics and prognostic factors.
Pozzi C, D'Amico M, Fogazzi GB, Curioni S, Ferrario F, Pasquali S, Quattrocchio G, Rollino C, Segagni S, Locatelli F
Am J Kidney Dis 2003 Dec;42(6):1154-63. doi: 10.1053/j.ajkd.2003.08.040. PMID: 14655186
The fibrillary glomerulopathies.
Korbet SM, Schwartz MM, Lewis EJ
Am J Kidney Dis 1994 May;23(5):751-65. doi: 10.1016/s0272-6386(12)70290-1. PMID: 8172222

Clinical prediction guides

Coexistence of amyloidosis and light chain deposition disease in the heart.
Sobue Y, Takemura G, Kawamura S, Yano T, Kanamori H, Morimoto SI, Matsuo H
Cardiovasc Pathol 2021 Mar-Apr;51:107315. Epub 2020 Nov 29 doi: 10.1016/j.carpath.2020.107315. PMID: 33264681
IgG Subclass Staining in Routine Renal Biopsy Material.
Hemminger J, Nadasdy G, Satoskar A, Brodsky SV, Nadasdy T
Am J Surg Pathol 2016 May;40(5):617-26. doi: 10.1097/PAS.0000000000000605. PMID: 26848798
Diffuse cystic lung diseases.
Ryu JH, Tian X, Baqir M, Xu K
Front Med 2013 Sep;7(3):316-27. Epub 2013 May 11 doi: 10.1007/s11684-013-0269-z. PMID: 23666611
Renal morphologic lesions reminiscent of diabetic nephropathy.
Raparia K, Usman I, Kanwar YS
Arch Pathol Lab Med 2013 Mar;137(3):351-9. doi: 10.5858/arpa.2012-0243-RA. PMID: 23451746
Light chain deposition disease with renal involvement: clinical characteristics and prognostic factors.
Pozzi C, D'Amico M, Fogazzi GB, Curioni S, Ferrario F, Pasquali S, Quattrocchio G, Rollino C, Segagni S, Locatelli F
Am J Kidney Dis 2003 Dec;42(6):1154-63. doi: 10.1053/j.ajkd.2003.08.040. PMID: 14655186

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