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Kidney Week

Abstract: PO2220

Sezary Syndrome with Renal Involvement

Session Information

  • Onco-Nephrology - 2
    October 22, 2020 | Location: On-Demand
    Abstract Time: 10:00 AM - 12:00 PM

Category: Trainee Case Report

  • 1500 Onco-Nephrology

Authors

  • Khalid, Sheikh Bilal, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States
  • Jennette, J. Charles, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States
  • Reynolds, Monica Lona, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States
  • Kanu, Obiajulu, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States
  • Singh, Vikas, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States
Introduction

Sezary Syndrome is a cutaneous T-cell lymphoma that presents with erythroderma, lymphadenopathy, and circulating malignant T cells. While involvement of the spleen, liver, bone marrow and lung are well documented, kidney involvement is rare. We present a case of acute kidney injury (AKI) due to biopsy-proven T cell lymphoma invasion of the kidneys

Case Description

A 69-year-old female with a history of cutaneous T-cell lymphoma was admitted to the oncology service with AKI. Serum creatinine (sCr) 3.3mg/dL (baseline 0.6mg/dL),urine protein-to-creatinine ratio 2.5g/g,urine sediment bland and renal ultrasound unremarkable.A PET scan, performed to evaluate systemic disease burden, revealed diffuse kidney enlargement with high FDG uptake throughout the renal parenchyma. A kidney biopsy was performed
Light microscopy showed diffuse interstitial infiltration by atypical small lymphoid cells and prominent focal apoptosis with apoptotic bodies and focal interstitial hemorrhage. The lymphoid cells had the same immunophenotype as the cutaneous T cell lymphoma
The glomeruli had slight segmental wrinkling of capillaries and glomerular basement membranes, and segmental podocyte swelling. She also had leptomeningeal involvement. Treatment included dexamethasone, systemic and intrathecal doxorubicin, methotrexate, and cytarabine. SCr returned to baseline. However, her course was complicated by severe mucositis, neutropenic fever, gastrointestinal hemorrhage and refractory shock. The patient was stabilized and opted to return home with hospice

Discussion

AKI caused by kidney involvement in Sezary syndrome has only been reported via case reports. The mechanism of AKI is thought to be tubular compression by the lymphomatous infiltrates impeding tubule function and peritubular capillary blood flow. Our patient’s biopsy supports this mechanism. Her AKI resolved with urgent corticosteroids and chemotherapy. Further reporting is needed on the prevalence of this condition and nephrologists should consider renal lymphomatous invasion when evaluating AKI in those with cutaneous T-cell lymphoma