Unraveling the Mystery: A Case of Cryoglobulinemia
- Glomerular Diseases: Case Reports
November 02, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Trials
- Pedapati, Shilpa, Banner University Medical Center Tucson, Tucson, Arizona, United States
- Nino, Jessica Maria, Banner University Medical Center Tucson, Tucson, Arizona, United States
- Rashid, Asma, Banner University Medical Center Tucson, Tucson, Arizona, United States
- González Negrete, Elvira, Banner University Medical Center Tucson, Tucson, Arizona, United States
- Mansour, Iyad S. M., Banner University Medical Center Tucson, Tucson, Arizona, United States
- Bracamonte, Erika R., Banner University Medical Center Tucson, Tucson, Arizona, United States
- Thajudeen, Bijin, Banner University Medical Center Tucson, Tucson, Arizona, United States
Cryoglobulinemia is a systemic inflammatory syndrome that generally involves small-to-medium vessel vasculitis due to cryoglobulin-containing immune complexes. The classification distinguishes three subtypes of cryoglobulinemic diseases. But at the same time, there are rare instances where there could be misleading classification of the disease due to the presence of two different underlying disorders. Here we report a case of type 2 cryoglobulinemic membranoproliferative glomerulonephritis with a detectable monoclonal IgM kappa in serum.
A 70-year-old gentleman with a past medical history of chronic myelogenous leukemia presented with epigastric abdominal pain and melena. He was diagnosed with liver cirrhosis and spontaneous bacterial peritonitis. Additional workup revealed active hepatitis C infections, acute kidney injury with hematuria, sub-nephrotic proteinuria, active sediment on urine microscopy, elevated RF, serum immunofixation showing monoclonal gammopathy, IgM, kappa type and urine immunofixation with lambda light chain band. A kidney Biopsy was performed that showed cryoglobulinemic glomerulonephritis. Due to monoclonal gammopathy, hematology performed a bone marrow biopsy, which returned 2-3% plasma cells. The diagnosis was felt to be more consistent with mixed/Type 2 Cryoglobulinemia secondary to HCV infection. The monoclonal gammopathy and the presence of Kappa-restricted B cells were felt to be an association rather than the cause of cryoglobulinemia. Therefore, he was not treated further with plasma cell-directed therapy. He received treatment with Rituximab, plasmapheresis, and steroids.
This case highlights the importance of differentiating between different types of cryoglobulinemia that determines appropriate therapy. Detectable monoclonal gammopathy can be found in the serum of patients with type II cryoglobulinemic glomerulonephritis. Although lymphoproliferative disorders are more closely related to type I cryoglobulinemia, it is essential to exclude monoclonal gammopathy in patients with mixed cryoglobulinemic glomerulonephritis to reveal hidden lymphoproliferative disease before the diagnosis of essential cryoglobulinemia can be made. This has implications for the management of such patients.