Abstract: FR-PO1246
Paraneoplastic Minimal Change Disease as an Expanded Spectrum of Monoclonal Gammopathy of Renal Significance in Chronic Lymphocytic Leukemia (CLL) with JAK2-Mutated Myeloproliferative Neoplasm
Session Information
- Onconephrology: Diagnostic Dilemmas, Therapy-Related Toxicities, and Clinical Cases
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Onconephrology
- 1600 Onconephrology
Authors
- Naseem, Anam, Mercy Fitzgerald Hospital, Darby, Pennsylvania, United States
- Yong, Stanley, Mercy Fitzgerald Hospital, Darby, Pennsylvania, United States
- Imam, Imtiaz, Mercy Fitzgerald Hospital, Darby, Pennsylvania, United States
- Rather, Manzoor, Mercy Fitzgerald Hospital, Darby, Pennsylvania, United States
Introduction
Monoclonal gammopathy of renal significance (MGRS) describes kidney injury driven by a B cell or plasma cell clone that does not treatment criteria for overt hematologic malignancy, and is classically associated with immune complex-mediated renal disease. Minimal change disease (MCD) is a podocytopathy typically considered idiopathic and steroid-responsive in adults. Evidence links MCD to lymphoproliferative disorders as a potential MGRS manifestation even without immune complex deposition. We report dialysis-dependent MCD in a patient with CLL and JAK2-mutated myeloproliferative neoplasm, highlighting steroid-refractory disease and the need for clone-directed therapy.
Case Description
A 77-year-old man with CLL, JAK2-mutated myeloproliferative neoplasm, rheumatoid arthritis and prior splenectomy presented with progressive anasarca and worsening kidney function. Baseline creatinine was 0.8 mg/dL; admission creatinine was 6.3 mg/dL. Urinalysis showed severe proteinuria (UPCR >1100 mg/g) without hematuria. ANA, ANCA, anti-GBM, hepatitis B/C, and HIV were negative. Hemodialysis was initiated for refractory AKI.
Renal biopsy showed MCD with 95% foot process effacement and superimposed acute tubular injury. Immunofluorescence showed no immune complex deposits along capillary walls. Bone marrow biopsy for worsening pancytopenia confirmed persistent JAK2 mutated myeloproliferative neoplasm and monoclonal B lymphocytosis. Despite high dose IV methylprednisolone followed by oral corticosteroids, the patient remained dialysis-dependent without renal recovery. Multidisciplinary evaluation supported a paraneoplastic podocytopathy within the MGRS spectrum, and clone-directed therapy with rituximab was recommended.
Discussion
This case highlights dialysis-dependent MCD in a patient with coexisting CLL and JAK2-mutated myeloproliferative neoplasm, supporting a clonal immune dysregulation driven pathogenesis. The absence of immune complex deposition and failure to respond to corticosteroids suggest a cytokine-mediated podocytopathy rather than classic immune complex MGRS. These findings expand the spectrum of MGRS to include non-immune complex MCD and underscore the importance of recognizing refractory cases in patients with hematologic disorders, where early clone-directed therapy such as rituximab may be required for renal recovery where corticosteroids alone are insufficient.