arrow-right-realarrow-right-whitearrow-rightback-to-topdoctordownloadfacebookinstagramlogo-markerlogo-wordmarkpodcastsearchsearch_whitetwitter
Close Modal

Blog


Currently filtering by tag: LCPT

Light Chain Proximal Tubulopathy Amyloid Proximal Tubulopathy Type

The two most common morphologic variants of light chain proximal tubulopathy (LCPT) are LCPT with and without crystal formation. Two additional rare variants can also be seen, namely amyloid proximal tubulopathy and LCPT with fibrillary aggregates. The histopathologic features of amyloid proximal tubulopathy are shown here. (A and B) Proximal tubules have scattered cytoplasmic inclusions (arrows) that stain blue on (A) Masson trichrome stain and are (B) Congo red positive (original magnification × 400). (C) The cytoplasmic inclusions show lambda restriction (arrow) by immunofluorescence (fluorescein-conjugated anti-human lambda; original magnification × 400). (D) Transmission electron photomicrograph showing an aggregate in the...

Light Chain Proximal Tubulopathy with Crystals

The two most common morphologic variants of light chain proximal tubulopathy (LCPT) are LCPT with and without crystal formation. The histopathologic lesions of light chain proximal tubulopathy with crystal formation are shown here. (A) A case with dense crystal deposition resulting in a focally pale appearance to proximal tubules on periodic acid-Schiff stain (original magnification × 100). (B) Crystals are easily identified due to dark staining on Toluidine blue stain (original magnification × 400). (C) A light chain crystal (arrow) stains brightly fuchsinophilic on Masson trichrome stain in this case with rare crystal deposition (original magnification × 400). (D-F) Crystals can...

Light Chain Proximal Tubulopathy

Light chain proximal tubulopathy (LCPT) is a disease characterized by the renal biopsy finding of cytoplasmic light chain restriction within the proximal tubule cells. It has traditionally been known for the presence of Fanconi syndrome though this is not present in the majority of cases in more recent case series.1,2 There is a morphologic spectrum to the disease. Most notably, it can occur with or without crystalline inclusions in the proximal tubule cytoplasm. The photomicrographs here show lambda restriction in the proximal tubule cytoplasm in a case without evidence of crystal formation by light and electron microscopy. When there is evidence...

Light Chain Proximal Tubulopathy Crystals

This image shows the prominent intracytoplasmic tubular epithelial crystals characteristic of a subset of light chain proximal tubulopathy cases associated with Fanconi syndrome (normoglycemic glycosuria, aminoaciduria, uricosuria, hyperphosphaturia with hypophosphatemia). The crystals represent precipitated nephrotoxic monoclonal light chains, and unlike cases without crystals, pronase-digested immunofluorescence may be helpful in proving light chain restriction when routine immunofluorescence is negative. Light chain proximal tubulopathy with crystals indicates an underlying clonal plasma cell proliferation, and may be seen in patients with monoclonal gammopathy of undetermined significance (MGUS), multiple myeloma, or lymphoma. Evidence of light chain cast nephropathy and/or monoclonal immunoglobulin deposition disease may also...

Light Chain Deposition Disease and Light Chain Proximal Tubulopathy

An elderly but previously healthy patient presents with weakness and fatigue and was found to have a creatinine of 3.0 mg/dl. His baseline Cr was 1.2 mg/dl four months prior. No history of diabetes or hypertension. Biopsy performed for kidney injury, acute versus chronic. Tubular Injury, interstitial fibrosis, and intact glomeruli. 1. Tubular Injury, interstitial fibrosis, and intact glomeruli 2. Linear staining for lambda light chains along tubular basement membranes 3. Negative staining for kappa light chains along tubular basement membranes 4. Positive Lambda staining in tubular droplets 5. Negative Kappa staining in tubular droplets Electron microscopy was negative for...

Diagnose This! (June 5, 2017)

What’s your diagnosis?     ​   ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​     ​   ​   ​ ​...