Non-crystalline light chain proximal tubulopathy, a morphologically protean entity.

ABSTRACT:

Background

Light chain proximal tubulopathy (LCPT) is a rare form of paraprotein-related disease, occurring in two main histopathological forms: crystalline and non-crystalline. The clinicopathological features, treatment strategies and outcomes, especially of the non-crystalline form, are not well described.

Methods

We conducted a single-centre retrospective case series of 12 LCPT patients, 5 crystalline and 7 non-crystalline, between 2005 and 2021.

Results

The median age was 69.5 years (range 47-80). Ten patients presented with CKD and significant proteinuria (median estimated glomerular filtration rate of 43.5 ml/min/1.73 m2; urine protein:creatinine ratio 328 mg/mmol). Only six patients had known haematological disease at the time of renal biopsy. Multiple myeloma

SUBMITTER: Kousios A 

PROVIDER: S-EPMC10615624 | biostudies-literature | 2023 Oct

REPOSITORIES: biostudies-literature