Background: Mantle cell lymphoma (MCL) accounts for 7% of adult mature B cell non-Hodgkin lymphomas (NHLs). Paraneoplastic focal segmental glomerulosclerosis (FSGS) is an exceedingly rare association of MCL. There is limited evidence on the outcomes of lymphoma associated paraneoplastic renal syndromes who are treated with Bruton tyrosine kinase inhibitors. We describe a rare case of MCL associated FSGS who was successfully treated with a chemotherapy free regimen of single agent ibrutinib and continues to be in sustained complete remission with respect to lymphoma and FSGS-related proteinuria at a follow-up of 2 years. Case Description: A 73-year-old gentleman presented with 2-month history of bilateral pedal oedema with frothy urine. Examination revealed generalized lymphadenopathy with hepatosplenomegaly. Twenty-four-hour urine protein was 11 grams/total volume, suggesting nephrotic syndrome. There were atypical circulating lymphocytes in the blood. Flowcytometry and cytogenetic studies from bone marrow aspirate and histopathology of lymph node biopsy confirmed the diagnosis of MCL. Kidney biopsy revealed FSGS. The patient was treated with ibrutinib 560 mg once daily considering his age and preference for oral therapy. At the end of two years of follow-up the patient is in complete remission for both MCL and FSGS. Conclusions: Our case highlights that FSGS can be a rare paraneoplastic renal manifestation of MCL. Bruton tyrosine kinase inhibitors can be safely used effectively in patients with paraneoplastic lymphoma associated FSGS.
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Bhattacharjee et al. (2023) studied this question.
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