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The morphologic spectrum and clinical significance of light chain proximal tubulopathy with and without crystal formation.
Mod Pathol. 2011 Nov; 24(11):1462-9.MP

Abstract

The renal diseases most frequently associated with myeloma include amyloidosis, monoclonal immunoglobulin deposition disease, and cast nephropathy. Less frequently reported is light chain proximal tubulopathy, a disease characterized by κ-restricted crystal deposits in the proximal tubule cytoplasm. Light chain proximal tubulopathy without crystal deposition is only loosely related to the typical light chain proximal tubulopathy, and little is known about this entity. A search was performed of the 10 081 native kidney biopsy samples processed by our laboratory over the past 2 years for cases that had light chain restriction limited to the proximal tubule cytoplasm. A total of 10 cases of light chain proximal tubulopathy without crystal deposition were found representing 3.1% of light chain-related diseases. Nine of these 10 showed λ-light chain restriction. Only three cases of light chain proximal tubulopathy with crystals were found accounting for 0.9% of light chain-related diseases. Two of these three were κ subtype. Plasma cell dyscrasia was unsuspected in seven of the 10 patients with light chain proximal tubulopathy without crystals at the time of renal biopsy. After the biopsy was reported, follow-up was available on 9/10 patients with 9/9 showing a plasma cell dyscrasia including 8/9 with multiple myeloma. We found that light chain proximal tubulopathy without crystal formation, despite being rarely described in the literature, is over three times more common than light chain proximal tubulopathy with crystal formation in our series. And given that it is often associated with previously unrecognized myeloma, it is a critically important diagnosis.

Authors+Show Affiliations

Nephropathology Associates, Little Rock, AR 72211, USA. Chris.larsen@nephropath.comNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

21701535

Citation

Larsen, Christopher P., et al. "The Morphologic Spectrum and Clinical Significance of Light Chain Proximal Tubulopathy With and Without Crystal Formation." Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc, vol. 24, no. 11, 2011, pp. 1462-9.
Larsen CP, Bell JM, Harris AA, et al. The morphologic spectrum and clinical significance of light chain proximal tubulopathy with and without crystal formation. Mod Pathol. 2011;24(11):1462-9.
Larsen, C. P., Bell, J. M., Harris, A. A., Messias, N. C., Wang, Y. H., & Walker, P. D. (2011). The morphologic spectrum and clinical significance of light chain proximal tubulopathy with and without crystal formation. Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc, 24(11), 1462-9. https://doi.org/10.1038/modpathol.2011.104
Larsen CP, et al. The Morphologic Spectrum and Clinical Significance of Light Chain Proximal Tubulopathy With and Without Crystal Formation. Mod Pathol. 2011;24(11):1462-9. PubMed PMID: 21701535.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The morphologic spectrum and clinical significance of light chain proximal tubulopathy with and without crystal formation. AU - Larsen,Christopher P, AU - Bell,Jane M, AU - Harris,Alexis A, AU - Messias,Nidia C, AU - Wang,Yihan H, AU - Walker,Patrick D, Y1 - 2011/06/24/ PY - 2011/6/25/entrez PY - 2011/6/28/pubmed PY - 2012/3/1/medline SP - 1462 EP - 9 JF - Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc JO - Mod. Pathol. VL - 24 IS - 11 N2 - The renal diseases most frequently associated with myeloma include amyloidosis, monoclonal immunoglobulin deposition disease, and cast nephropathy. Less frequently reported is light chain proximal tubulopathy, a disease characterized by κ-restricted crystal deposits in the proximal tubule cytoplasm. Light chain proximal tubulopathy without crystal deposition is only loosely related to the typical light chain proximal tubulopathy, and little is known about this entity. A search was performed of the 10 081 native kidney biopsy samples processed by our laboratory over the past 2 years for cases that had light chain restriction limited to the proximal tubule cytoplasm. A total of 10 cases of light chain proximal tubulopathy without crystal deposition were found representing 3.1% of light chain-related diseases. Nine of these 10 showed λ-light chain restriction. Only three cases of light chain proximal tubulopathy with crystals were found accounting for 0.9% of light chain-related diseases. Two of these three were κ subtype. Plasma cell dyscrasia was unsuspected in seven of the 10 patients with light chain proximal tubulopathy without crystals at the time of renal biopsy. After the biopsy was reported, follow-up was available on 9/10 patients with 9/9 showing a plasma cell dyscrasia including 8/9 with multiple myeloma. We found that light chain proximal tubulopathy without crystal formation, despite being rarely described in the literature, is over three times more common than light chain proximal tubulopathy with crystal formation in our series. And given that it is often associated with previously unrecognized myeloma, it is a critically important diagnosis. SN - 1530-0285 UR - https://www.unboundmedicine.com/medline/citation/21701535/The_morphologic_spectrum_and_clinical_significance_of_light_chain_proximal_tubulopathy_with_and_without_crystal_formation_ L2 - http://dx.doi.org/10.1038/modpathol.2011.104 DB - PRIME DP - Unbound Medicine ER -