Kidney Week

Abstract: PO2211

Iron Overload Syndrome and Primary Focal Segmental Glomerulosclerosis Recurrent with Monthly Plasma Exchange Therapy: Long-Term Second Kidney Allograft Survival

Session Information

• Transplantation: Clinical - Noninvasive Biomarkers, Immune Regulation, and Fascinomas
  November 04, 2021 | Location: On-Demand, Virtual Only
  Abstract Time: 10:00 AM - 12:00 PM

Category: Transplantation

• 1902 Transplantation: Clinical

Authors

• Martinez-Vazquez, Belen, Instituto Nacional de Cardiologia Ignacio Chavez, Mexico, ciudad de Mexico, Mexico

Belen Martinez-Vazquez, MD

• Gudiño Bravo, Pedro, Instituto Nacional de Cardiologia Ignacio Chavez, Mexico, ciudad de Mexico, Mexico

Pedro Gudiño Bravo,

• Garcia-Flores, Octavio Rene, Instituto Nacional de Cardiologia Ignacio Chavez, Mexico, ciudad de Mexico, Mexico

Octavio Rene Garcia-Flores,

• Rodríguez, Francisco, Instituto Nacional de Cardiologia Ignacio Chavez, Mexico, ciudad de Mexico, Mexico

Francisco Rodríguez,

Introduction

Patients who lose their allograft due to recurrent FSGS are usually not retransplanted since the risk of recurrence (80–100%). Proteinuria as a result of glomerular damage is linked to tubulointerstitial injury, which is associated with increased filtration of transferrin-bound iron and can lead to tubular accumulation.

Case Description

A 32-year old female with a history of collapsing FSGS, living related kidney transplant with loss of the allograft function due to recurrent disease underwent a 2^nd kidney transplantation in October 2018. At 2 months post transplantation elevated levels of proteinuria (6 g/g), kidney biopsy (KB) demonstrated recurrent FSGS. She received treatment with high-dose steroids, CsA and an intensive course of plasma exchanges (PEs) due to persistent proteinuria. There is no history of blood transfusions, iron treatment and diseases with ineffective erythropoiesis. Ferritin levels (15000 ng/ml) and a MRI with liver and spleen iron deposition pointed to the diagnosis of Hereditary Hemochromatosis (HH), common mutations (C282Y, H63D and G320V genes) were negative. KB of 2020 demonstrated iron deposition (ID) in tubular epithelial cells.

Discussion

Histological evidence of ID in the tubulointerstitium can be related to persistent proteinuria. The negative genetic testing is common in the Hispanic race in which there is a lesser prevalence for the most frequent mutation, C282Y homozygosity (0.03% compared to 0.44% in Whites). We assume that this patient has a genetically unknown type of HH. To our knowledge this is the first reported case in which an Iron Overload Syndrome (IOS) is associated with FSGS. PEs is a therapeutic option in patients with recurrent FSGS, also used in IOS.