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Published on: May 22, 2020
Minimal Change Nephrotic Syndrome Complicated by Classical Hodgkin Lymphoma During Prolonged Rituximab Maintenance
Koshi Yamada1, Mayu Kijiya1, Takeo Koshida1
1Department of Nephrology, Juntendo University Faculty of Medicine, Tokyo, Japan.
Minimal change nephrotic syndrome (MCNS) has traditionally been linked to T-cell dysfunction; however, mounting evidence suggests that B-cell-mediated mechanisms also play a significant role. Rituximab (RTX) is increasingly utilized as maintenance therapy for recurring MCNS, yet its extended safety profile remains uncertain. In this study, we report a rare case of MCNS complicated by classical Hodgkin lymphoma (cHL) that emerged during prolonged RTX maintenance therapy. A 23-year-old woman with relapsing MCNS achieved sustained remission with RTX maintenance therapy. Following 13 RTX courses, the patient developed nodular sclerosis-type cHL and attained complete remission with A + AVD chemotherapy (adriamycin, brentuximab vedotin, vinblastine, and dacarbazine). Despite successful chemotherapy for cHL, the patient relapsed with nephrotic syndrome featuring significant proteinuria (8.43 g/day) as RTX efficacy declined. This clinical progression illustrates a close temporal link between RTX effect loss and disease recurrence, supporting the contributory role of elevated peripheral CD19+ B cells in MCNS relapse. Although sustained B-cell-targeted therapy seems effective for prolonged disease management, the exceedingly rare incidence of cHL during extended RTX maintenance in this patient highlights the importance of vigilant long-term safety monitoring with prolonged B-cell depletion.
Minimal change nephrotic syndrome (MCNS) has traditionally been linked to T-cell dysfunction; however, mounting evidence suggests that B-cell-mediated mechanisms also play a significant role. Rituximab (RTX) is increasingly utilized as maintenance therapy for recurring MCNS, yet its extended safety profile remains uncertain. In this study, we report a rare case of MCNS complicated by classical Hodgkin lymphoma (cHL) that emerged during prolonged RTX maintenance therapy. A 23-year-old woman with relapsing MCNS achieved sustained remission with RTX maintenance therapy. Following 13 RTX courses, the patient developed nodular sclerosis-type cHL and attained complete remission with A + AVD chemotherapy (adriamycin, brentuximab vedotin, vinblastine, and dacarbazine). Despite successful chemotherapy for cHL, the patient relapsed with nephrotic syndrome featuring significant proteinuria (8.43 g/day) as RTX efficacy declined. This clinical progression illustrates a close temporal link between RTX effect loss and disease recurrence, supporting the contributory role of elevated peripheral CD19+ B cells in MCNS relapse. Although sustained B-cell-targeted therapy seems effective for prolonged disease management, the exceedingly rare incidence of cHL during extended RTX maintenance in this patient highlights the importance of vigilant long-term safety monitoring with prolonged B-cell depletion.
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