Minimal change nephropathy and IgA deposits associated with a Sezary syndrome

• Published in: La revue de medecine interne 2022-01, Vol.43 (1), p.48-53
• Main Authors: Bichon, A, Brue, A, Godefroy, R, Sallee, M, Daniel, L, Farnarier, C, Gobin, N, Abed, S, Richard, M A, Villani, P, Malissen, N, Daumas, A
• Format: Article
• Language: fre
• Subjects: Aged, 80 and over; Female; Glomerulonephritis, IGA - complications; Glomerulonephritis, IGA - diagnosis; Humans; Immunoglobulin A; Nephrosis, Lipoid - complications; Nephrosis, Lipoid - diagnosis; Nephrotic Syndrome - complications; Nephrotic Syndrome - diagnosis; Sezary Syndrome - complications; Sezary Syndrome - diagnosis; Skin Neoplasms - complications; Skin Neoplasms - diagnosis
• ISSN: 1768-3122
• DOI: 10.1016/j.revmed.2021.08.006

The Sézary syndrome (SS) is an aggressive form of cutaneous T-cell lymphoma (CTCL) requiring a rapid diagnosis due to its poor prognosis. We report the first case of an eighty-nine-year-old woman who presented with concomitant Sezary syndrome and anasarca, revealing a nephrotic syndrome caused by a minimal change nephropathy associated with immunoglobulin A (IgA) deposits. Scarce literature described rare cases associating these two entities (nephrotic syndrome and nephropathy). However, the nephrotic syndrome was delayed from disease onset, secondary to immunosuppressive treatment of SS, or due to the weaning of SS therapy. Thus, the direct link between the glomerular lesion and the cutaneous lymphoma was difficult to establish. However, the synchronous occurrence of both SS and glomerulopathy in our patient, along with Sezary cells in both urines (urinary cytology) and biopsy, and resolution of nephropathy after treatment of SS, support the likely attributability of SS in glomerulopathy. Practitioners must acknowledge the possible occurrence of glomerular involvement in SS.