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Am J Kidney Dis. 2018 Dec;72(6):890-894. doi: 10.1053/j.ajkd.2018.04.013. Epub 2018 Jun 22.

Sézary Syndrome Presenting With Renal Involvement.

Author information

1
Department of Haematology, University Hospitals of Leicester NHS Trust, Leicester, United Kingdom.
2
John Walls Renal Unit, University Hospitals of Leicester NHS Trust, Leicester, United Kingdom; Department of Infection, Immunity and Inflammation, University of Leicester, Leicester, United Kingdom. Electronic address: mgb23@le.ac.uk.
3
Department of Dermatology, University Hospitals of Leicester NHS Trust, Leicester, United Kingdom.
4
Department of Histopathology, University Hospitals of Leicester NHS Trust, Leicester, United Kingdom.
5
Department of Infection, Immunity and Inflammation, University of Leicester, Leicester, United Kingdom.
6
John Walls Renal Unit, University Hospitals of Leicester NHS Trust, Leicester, United Kingdom.
7
John Walls Renal Unit, University Hospitals of Leicester NHS Trust, Leicester, United Kingdom; Department of Infection, Immunity and Inflammation, University of Leicester, Leicester, United Kingdom.

Abstract

Sézary syndrome is a rare aggressive leukemic variant of primary cutaneous T-cell lymphoma, typically presenting with erythroderma, lymphadenopathy, and an atypical clonal T-cell population. Though it often involves the spleen and liver, we report a case of Sézary syndrome with renal involvement that was treated successfully. Visceral involvement confers a poor prognosis requiring systemic treatment. The patient we describe was a 66-year-old man who was referred from Dermatology services for deteriorating kidney function. Polymerase chain reaction of genomic DNA from skin and kidney biopsies confirmed a clonal T-cell population matching a population isolated in peripheral blood. The patient was treated initially with alemtuzumab, which led to a significant improvement in kidney function, and he has subsequently received a successful allogeneic stem cell transplant. This case represents a rare cause of decreased kidney function and highlights the role of biopsy in patients with suspected Sézary syndrome.

KEYWORDS:

Sézary syndrome; acute kidney injury (AKI); case report; cutaneous T-cell lymphoma; erythroderma; kidney biopsy; kidney function; lymphocytosis

PMID:
29941220
DOI:
10.1053/j.ajkd.2018.04.013
[Indexed for MEDLINE]

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