Tuesday, January 20, 2015

Topic Discussion: Proximal Tubulopathies with monoclonal light chains

Proximal tubule is a commonly involved part of the kidney in myeloma.  Classically, they are noted more with light chains compared to heavy chains.  A recent article sheds light on the four mechanisms on how they might impact the proximal tubule and lead to four distinct clinical settings. I have summarized the four types in a table format here.

Proximal tubulopathy without cytoplasmic inclusions
Proximal tubulopathy with interstitial inflammation(AIN variant)
Proximal tubulopathy with cytoplasmic inclusions
Proxmial tubulopathy with lysosomal indigestion
Vacuolization of tubular cells and perhaps some necrosis
Vacuolization but AIN as well. Lymphocytes mainly
Swollen proximal tubular cells and EM shows rectangular or angulated inclusions in cytoplasm appear crystalline in nature
Enlarged proximal cells, EM shows lysosome was occupied by substance.
Predominant light chain
Kappa more than lambda
Kappa more than lambda
Kappa and one case of lambda
All kappa
Seen with MGUS
Slowly progressive renal failure, some non nephrotic proteinuria
ARF, more patients requiring dialysis, non nephrotic proteinuria
Slowly progressive renal failure,glucosuria, phosphaturia and non nephrotic range proteinuria
Slowly progressive, glucosuria and phosphaturia
Unique features
Second most prevalent, older age
Most prevalent, older age
Younger age
Least prevalent, younger age

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