Understanding kidney involvement in mycosis fungoides: T-cell clonality as a guide for targeted therapy – a case report and literature review
摘要
Mycosis fungoides (MF) is a common T-cell lymphoma that primarily affects the skin. Renal involvement is rare and has never been reported as the initial extracutaneous site. T-cell clonality testing is essential for confirming systemic involvement. We report identical T-cell clonality in both the skin and renal involvement of MF, accompanied by a review of the literature on MF involvement in the kidneys.
Case presentationA 58-year-old man with folliculotropic MF had asymptomatic bilateral kidney lesions incidentally detected on a routine magnet resonance imaging (MRI) 15 years after primary diagnosis. Immunohistochemistry (IHC) and polymerase chain reaction (PCR) confirmed clonal T-cell populations in skin and kidney biopsies, verifying systemic involvement. A Positron Emission Tomography (PET) scan showed a 50% reduction in kidney lesions after four months of therapy with Liposomal doxorubicin (20 mg/m2). However, despite this initial response, the disease spread to the lungs and pancreas, and the patient passed away eight months after kidney infiltration.
ConclusionThis is the first documented confirmation of MF involvement to the kidneys through specific IHC and T-cell PCR-confirmed clonality testing. It highlights advances in therapy for localized disease and underscores the importance of confirming T-cell clonality, especially in atypical sites like the kidneys, illustrating its potential to enhance targeted therapy in disseminated MF.