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Light Chain Proximal Tubulopathy without Crystals

MedGen UID:
1700527
•Concept ID:
C5204118
•
Disease or Syndrome
Synonym: Proximal Tubulopathy without Crystals

Definition

A morphologic variant of light chain proximal tubulopathy with no evidence of crystal formation. It is characterized by acute tubular injury with no evidence lysosomal immunoglobulin crystals. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLight Chain Proximal Tubulopathy without Crystals

Professional guidelines

PubMed

Light Chain Proximal Tubulopathy: Clinical and Pathologic Characteristics in the Modern Treatment Era.
Stokes MB, Valeri AM, Herlitz L, Khan AM, Siegel DS, Markowitz GS, D'Agati VD
J Am Soc Nephrol 2016 May;27(5):1555-65. Epub 2015 Sep 15 doi: 10.1681/ASN.2015020185. PMID: 26374607Free PMC Article

Recent clinical studies

Diagnosis

Improvement of Light Chain Proximal Tubulopathy without Crystals in IgGλ-type Monoclonal Gammopathy of Undetermined Significance Using Bortezomib and Dexamethasone.
Tsuyuki T, Uramatsu T, Shimizu M, Ishi T, Tsuji K, Nakashima J, Katafuchi E, Nakayama T, Uesugi N, Muta K, Nishino T
Intern Med 2024 Mar 1;63(5):693-698. Epub 2023 Jul 12 doi: 10.2169/internalmedicine.1835-23. PMID: 37438138Free PMC Article
Light chain proximal tubulopathy without crystals in a case of Burkitt lymphoma presenting with acute kidney injury.
Rane S, Rana S, Sachdeva M, Joshi K
Am J Kidney Dis 2013 Sep;62(3):638-41. Epub 2013 Mar 22 doi: 10.1053/j.ajkd.2013.01.026. PMID: 23523237
The morphologic spectrum and clinical significance of light chain proximal tubulopathy with and without crystal formation.
Larsen CP, Bell JM, Harris AA, Messias NC, Wang YH, Walker PD
Mod Pathol 2011 Nov;24(11):1462-9. Epub 2011 Jun 24 doi: 10.1038/modpathol.2011.104. PMID: 21701535

Therapy

Improvement of Light Chain Proximal Tubulopathy without Crystals in IgGλ-type Monoclonal Gammopathy of Undetermined Significance Using Bortezomib and Dexamethasone.
Tsuyuki T, Uramatsu T, Shimizu M, Ishi T, Tsuji K, Nakashima J, Katafuchi E, Nakayama T, Uesugi N, Muta K, Nishino T
Intern Med 2024 Mar 1;63(5):693-698. Epub 2023 Jul 12 doi: 10.2169/internalmedicine.1835-23. PMID: 37438138Free PMC Article

Clinical prediction guides

Improvement of Light Chain Proximal Tubulopathy without Crystals in IgGλ-type Monoclonal Gammopathy of Undetermined Significance Using Bortezomib and Dexamethasone.
Tsuyuki T, Uramatsu T, Shimizu M, Ishi T, Tsuji K, Nakashima J, Katafuchi E, Nakayama T, Uesugi N, Muta K, Nishino T
Intern Med 2024 Mar 1;63(5):693-698. Epub 2023 Jul 12 doi: 10.2169/internalmedicine.1835-23. PMID: 37438138Free PMC Article

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