Introduction: As a very rare form of B-cell lymphoma, plasmablastic lymphoma (PBL) typically occurs in patients with underlying immunosuppression, including human immunodeficiency virus (HIV), organ transplantation, and autoimmune diseases. For HIV-positive patients, PBL normally originates in the gastrointestinal tract, especially from the oral cavity in most cases. It is extremely rare to find abdominal cavity involvement in PBL, and there has been no previously reported instance of proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) attributed to monoclonal IgG (MIgG) lambda secreted by PBL.
Case Presentation: We report the case of an HIV-negative female with nephrotic syndrome, renal insufficiency, and multiple swollen lymph nodes. Ascitic fluid cytology revealed a high level of plasmablast-like lymphocytes with the restriction of lambda light chains. Besides, the renal biopsy revealed PGNMID, which could presumably be secondary to MIgG-lambda-secreting by PBL. MIgG-lambda-restricted expression was discovered earlier in the kidney tissue than in the blood.
Conclusion: The diagnostic landscape for PBL is notoriously intricate, necessitating a multifaceted and nuanced approach to mitigate the risks of erroneous identification.
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http://dx.doi.org/10.1186/s12882-023-03351-7 | DOI Listing |
Tunis Med
December 2024
Research Laboratory LR18/SP12 "Autoimmunity, Cancer, and Immunogenetics", Habib Bourguiba hospital, Sfax, Tunisia.
Introduction: Lupus nephritis (LN) is an immune complex glomerulonephritis, caused by systemic lupus erythematosus. It is associated with an increase of morbidity and mortality. In LN, the immune responses dysregulation is one of the crucial pathogenic pathways.
View Article and Find Full Text PDFClin Med Insights Case Rep
December 2024
Department of Nephrology and Endocrinology, National Defense Medical College, Tokorozawa, Japan.
This study reports a rare case of immune-complex mediated mesangial proliferative glomerulonephritis (ICGN) with a full-house pattern in a 56-year-old Japanese man, observed during the treatment of immune thrombocytopenic purpura (ITP). Because of persistent complement deficiency and worsening of kidney function, he was treated with prednisolone, and his urinary findings improved markedly. However, as the complement titers were still low, mycophenolate mofetil was also prescribed, which normalized complement levels.
View Article and Find Full Text PDFDiagnostics (Basel)
November 2024
Division of Nephrology, Hospital das Clínicas, University of São Paulo School of Medicine, São Paulo 05403-010, SP, Brazil.
Background: Since the introduction of the SLICC criteria in 2012, biopsy-proven lupus nephritis (LN) has been the only independent diagnostic criterion for systemic lupus erythematosus (SLE). This was reaffirmed by the EULAR/ACR in 2019, emphasizing the importance of renal biopsy in LN. However, the current classification lacks specific histopathological criteria for defining LN.
View Article and Find Full Text PDFCEN Case Rep
December 2024
Department of Nephrology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, 1678 Dongfang Road, Shanghai, China.
Secondary vasculitis is encountered in about one-third of all cases of systemic lupus erythematosus (SLE). Skin is most commonly involved in lupus-related small vasculitis. Although antineutrophil cytoplasmic autoantibodies (ANCA) associated vasculitis (AAV) is relatively uncommon, it can be the most dangerous manifestation associated with high mortality.
View Article and Find Full Text PDFIndian J Nephrol
June 2024
Department of Nephrology, Setubal Hospital Center, Setúbal, Portugal.
C3 glomerulonephritis (C3GN) is rare. It is a proliferative glomerulonephritis resulting from glomerular deposition of complement factors due to dysregulation of the alternative pathway of complement. The association between monoclonal protein production and development of C3GN was described.
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