Abstract: TH-PO956
Favorable Effect of Bortezomib in Patients with Noninfectious Mixed Cryoglobulinemia Complicated with B Cell Lymphoma: Experience of Two Cases
Session Information
- Glomerular Trainee Case Reports
November 07, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Report
- 1202 Glomerular Diseases: Immunology and Inflammation
Author
- Ueda, Yusho, Hokkaido University, Sapporo, Japan
Introduction
Non-infectious mixed cryoglobulinemia (MC) is rare disorder associated with autoimmune or hematological diseases, eventually causing systemic vasculitis with organ damage. Due to the low frequency of this disease, the therapeutic strategy for non-infectious MC has not been established. Here we report that two cases with non-infectious MC complicated with B cell lymphoma who had good response to a combination of corticosteroid and bortezomib (BTZ), a proteasome inhibitor used for the treatment of multiple myeloma and B cell Lymphoma.
Case Description
Case1. A 61-year-old man with a medical history of mucosa-associated lymphoid tissue (MALT) lymphoma presented with nephrotic syndrome, purpura, and congestive heart failure. Laboratory tests revealed monoclonal IgM-κ and presence of cryoglobulins (CG). A renal biopsy showed glomerulonephritis with the deposits of IgM-κ. A diagnosis of non-infectious cryoglobulinemic glomerulonephritis was made and treatment was initiated with corticosteroid, rituximab and plasma exchange with no improvement of clinical manifestations. After the informed concent, the patient received BTZ in combination with corticosteroid, leading to amelioration of his clinical manifestations. Case2. A 69-year-old man presented with dyspnea due to severe nephrosis. Serum monoclonal IgM-κ and CG were detected. A bone marrow biopsy revealed low-grade B-cell lymphoma. He was diagnosed with cryoglobulinemic glomerulonephritis due to non-infectious MC complicated with B cell lymphoma. He was treated with a combination therapy of BTZ with corticosteroid, and achieved clinical remission of nephrotic syndrome.
Discussion
We presented two case of non-infectious MC complicated with lymphoma in whom BTZ was added in the treatment protocol to ameliorate renal manifestations. The improvement of renal function was associated with the disappearance of serum CG and decrease of serum levels of monoclonal IgM-κ. These findings suggest that BTZ suppresses the IgM-κ producing plasma cells and the deposition of CG in glomeruli, leading to the improvement of nephrosis. BTZ had anti-inflammatory effects as well, presumably involved in the protection of renal injury. This is a first report to show that treatment protocol including BTZ lead favourable outcome in patients with non-infectious MC with B cell lymphoma.