Crystals, crystals everywhere but not a clue till late… Light chain crystalline proximal tubulopathy with concomitant myeloma cast nephropathy

The renal diseases commonly associated with myeloma include primary amyloidosis, cast nephropathy, and light chain deposition disease. Less frequent forms of renal involvement encountered in the course of myeloma are crystalline and non-crystalline proximal tubulopathies, neoplastic plasma cell infi...

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Main Authors: Smita Mary Matthai, Suceena Alexander, Shibu Jacob, Neelaveni Duhli, Vinoi George David, Santosh Varughese
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2020-01-01
Series:Indian Journal of Pathology and Microbiology
Subjects:
Online Access:http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2020;volume=63;issue=3;spage=463;epage=466;aulast=Matthai
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spelling doaj-798b1191f40c40eab5badc4d71cd2cd72020-11-25T03:54:30ZengWolters Kluwer Medknow PublicationsIndian Journal of Pathology and Microbiology0377-49292020-01-0163346346610.4103/IJPM.IJPM_756_18Crystals, crystals everywhere but not a clue till late… Light chain crystalline proximal tubulopathy with concomitant myeloma cast nephropathySmita Mary MatthaiSuceena AlexanderShibu JacobNeelaveni DuhliVinoi George DavidSantosh VarugheseThe renal diseases commonly associated with myeloma include primary amyloidosis, cast nephropathy, and light chain deposition disease. Less frequent forms of renal involvement encountered in the course of myeloma are crystalline and non-crystalline proximal tubulopathies, neoplastic plasma cell infiltration, and immunoglobulin crystallization in interstitial histiocytes and glomerular cells including podocytes. Light chain proximal tubulopathy (LCPT) caused by aggregation of non-crystalline and rarely crystalline deposits of monoclonal light chains in the cytoplasm of proximal tubular epithelial cells, accounts for less than 5% of monoclonal gammopathy-associated kidney diseases. We report the case of a 48-year-old Indian woman with multiple myeloma, who presented with acute kidney injury and nephrotic syndrome, in whom the renal biopsy revealed widespread crystalline inclusions in extraglomerular and glomerular compartments. We present illustrative light microscopic (LM) and diagnostic electron microscopic (EM) findings of this case which enabled a diagnosis of crystalline LCPT, crystal storing histiocytosis, and crystalline podocytopathy occurring synchronously with myeloma cast nephropathy. While documenting this unique juxtapositioning of multicompartmental paraproteinemic renal injury in multiple myeloma, diagnosed after EM analysis of the patient's renal biopsy, we discuss the pathogenetic pathways of this condition along with the clinical implications. Due to intrinsic structural properties of the crystals, they frequently escape detection by routine LM, necessitating EM analysis for their diagnosis. Given the prognostic implications of tubulopathies complicating myeloma, LCPT is a critically important diagnosis, highlighting the need for a comprehensive renal biopsy evaluation inclusive of EM for the practice of precision medicine in such scenarios.http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2020;volume=63;issue=3;spage=463;epage=466;aulast=Matthaiacute kidney injurycrystalline tubulopathycrystal storing histiocytosiscrystalline podocytopathyfanconi's anemialight chain deposition diseasemyeloma kidneymyeloma cast nephropathy
collection DOAJ
language English
format Article
sources DOAJ
author Smita Mary Matthai
Suceena Alexander
Shibu Jacob
Neelaveni Duhli
Vinoi George David
Santosh Varughese
spellingShingle Smita Mary Matthai
Suceena Alexander
Shibu Jacob
Neelaveni Duhli
Vinoi George David
Santosh Varughese
Crystals, crystals everywhere but not a clue till late… Light chain crystalline proximal tubulopathy with concomitant myeloma cast nephropathy
Indian Journal of Pathology and Microbiology
acute kidney injury
crystalline tubulopathy
crystal storing histiocytosis
crystalline podocytopathy
fanconi's anemia
light chain deposition disease
myeloma kidney
myeloma cast nephropathy
author_facet Smita Mary Matthai
Suceena Alexander
Shibu Jacob
Neelaveni Duhli
Vinoi George David
Santosh Varughese
author_sort Smita Mary Matthai
title Crystals, crystals everywhere but not a clue till late… Light chain crystalline proximal tubulopathy with concomitant myeloma cast nephropathy
title_short Crystals, crystals everywhere but not a clue till late… Light chain crystalline proximal tubulopathy with concomitant myeloma cast nephropathy
title_full Crystals, crystals everywhere but not a clue till late… Light chain crystalline proximal tubulopathy with concomitant myeloma cast nephropathy
title_fullStr Crystals, crystals everywhere but not a clue till late… Light chain crystalline proximal tubulopathy with concomitant myeloma cast nephropathy
title_full_unstemmed Crystals, crystals everywhere but not a clue till late… Light chain crystalline proximal tubulopathy with concomitant myeloma cast nephropathy
title_sort crystals, crystals everywhere but not a clue till late… light chain crystalline proximal tubulopathy with concomitant myeloma cast nephropathy
publisher Wolters Kluwer Medknow Publications
series Indian Journal of Pathology and Microbiology
issn 0377-4929
publishDate 2020-01-01
description The renal diseases commonly associated with myeloma include primary amyloidosis, cast nephropathy, and light chain deposition disease. Less frequent forms of renal involvement encountered in the course of myeloma are crystalline and non-crystalline proximal tubulopathies, neoplastic plasma cell infiltration, and immunoglobulin crystallization in interstitial histiocytes and glomerular cells including podocytes. Light chain proximal tubulopathy (LCPT) caused by aggregation of non-crystalline and rarely crystalline deposits of monoclonal light chains in the cytoplasm of proximal tubular epithelial cells, accounts for less than 5% of monoclonal gammopathy-associated kidney diseases. We report the case of a 48-year-old Indian woman with multiple myeloma, who presented with acute kidney injury and nephrotic syndrome, in whom the renal biopsy revealed widespread crystalline inclusions in extraglomerular and glomerular compartments. We present illustrative light microscopic (LM) and diagnostic electron microscopic (EM) findings of this case which enabled a diagnosis of crystalline LCPT, crystal storing histiocytosis, and crystalline podocytopathy occurring synchronously with myeloma cast nephropathy. While documenting this unique juxtapositioning of multicompartmental paraproteinemic renal injury in multiple myeloma, diagnosed after EM analysis of the patient's renal biopsy, we discuss the pathogenetic pathways of this condition along with the clinical implications. Due to intrinsic structural properties of the crystals, they frequently escape detection by routine LM, necessitating EM analysis for their diagnosis. Given the prognostic implications of tubulopathies complicating myeloma, LCPT is a critically important diagnosis, highlighting the need for a comprehensive renal biopsy evaluation inclusive of EM for the practice of precision medicine in such scenarios.
topic acute kidney injury
crystalline tubulopathy
crystal storing histiocytosis
crystalline podocytopathy
fanconi's anemia
light chain deposition disease
myeloma kidney
myeloma cast nephropathy
url http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2020;volume=63;issue=3;spage=463;epage=466;aulast=Matthai
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