Cryoglobulinemic vasculitis and monoclonal gammopathy in end-stage renal disease
نویسندگان
چکیده
which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. A 75-year-old man with a 2-year history of end-stage renal disease had skin lesions on both feet (A) for 2 weeks. The cause of end-stage renal disease remained to be clarified. Initial laboratory tests showed the following values: extracellular aggregates of pink-tan acellular material (B). We suspected cryoglobulinemia and performed the cryoglobulin test. The sampling was performed using a warmed syringe and a test tube. The patient was positive with a very high cryocrit level (37.02%). Immunofixation showed a monoclonal IgG kappa band in the gamma region. The patient was negative for hepatitis and HIV. Biopsy of the skin lesion showed leukocytoclastic vasculitis with subcorneal bulla. A subsequent bone marrow (BM) examination showed no evidence of clonal plasma cells (<2%). Flow cytometric analyses showed increased proportion of B cells (PB, 24.4%; BM aspirates, 25.5%) with no clonality. Cytogenetic study revealed normal karyotype of 46,XY. The patient was diagnosed with type I cryoglobulinemia with cryoglobulinemic vasculitis and monoclonal gammopathy. Treatment with methyl prednisolone improved his clinical response. Our data indicate that cryoglobulin could be easily detected by PB smear.
منابع مشابه
Successful Treatment of Monoclonal Gammopathy of Renal Significance With Bortezomib in the Setting of Post- Viral SARs-CoV-2 Infection: Case Report
Introduction: Monoclonal Gammopathy of Renal Significance (MGRS) is an immunoglobulin proliferative disorder that leads to the destruction of the renal glomerular basement membrane and progression to end-stage renal disease. The pathogenesis of MGRS is similar to that of multiple myeloma and chronic lymphocytic lymphoma but lacks criteria for either disease. This inability to characterize the d...
متن کاملBortezomib in type I cryoglobulinemic vasculitis: are we acting too late?
Type II and type III cryoglobulinemic vasculitis (CV) are characterized by a deranged immune function due to concomitant chronic infections or rheumatic disorders. Conversely, type I CV is caused by plasma cell dyscrasia. Bortezomib is a proteasome inhibitor that is largely employed as a first-line treatment for multiple myeloma. The use of bortezomib in cases of monoclonal gammopathy of undete...
متن کاملWaldenstrom Macroglobulinemia with CD5+ Expression Presented as Cryoglobulinemic Glomerulonephropathy: A Case Report
Waldenstrom macroglobulinemia (WM) is a B-cell lymphoproliferative disorder associated with bone marrow involvement of lymphoplasmacytic lymphoma (LPL) and an IgM monoclonal gammopathy. Generally B-lymphocytes in LPL do not express CD5 that is important for differential diagnosis of B-cell lymphoproliferative disorders. In WM, various renal diseases and type I cryoglobulinemia are well describe...
متن کاملA Patient with Abnormal Kidney Function and a Monoclonal Light Chain in the Urine.
Monoclonal gammopathy is increasingly recognized as a cause of kidney injury. These renal conditions behave differently than ones without monoclonal gammopathy and require specific treatment. To avoid misdiagnosis, testing for paraprotein should be performed in addition to vasculitis and autoimmune diseases serologies in adults with unexplained AKI or proteinuria. Because the prevalence of mono...
متن کاملThe challenge of treating hepatitis C virus-associated cryoglobulinemic vasculitis in the era of anti-CD20 monoclonal antibodies and direct antiviral agents
Mixed cryoglobulinemia syndrome (MC) is a systemic vasculitis involving kidneys, joints, skin, and peripheral nerves. While many autoimmune, lymphoproliferative, and neoplastic disorders have been associated with this disorder, hepatitis C virus (HCV) is known to be the etiologic agent in the majority of patients. Therefore, clinical research has focused on anti-viral drugs and, more recently, ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
دوره 46 شماره
صفحات -
تاریخ انتشار 2011