ABSTRACT
Objective@#To investigate the clinical significance and pathological features of lymphocytes and plasma cells infiltration and related ectopic lymphoid-like structures in IgG4-related tubulointerstitial nephritis (IgG4-TIN).@*Methods@#Complete data was collected from 24 patients with IgG4-TIN confirmed by pathology in the Peking University First Hospital. The renal specimens were examined by routine light microscopy, immunofluorescence and electron microscopy examination. In addition, immunohistochemistry was used to detect the distribution of CD20+ B lymphocytes, CD3+ T lymphocytes and CD138+ plasma cells.@*Results@#A total of 24 patients were enrolled in the study, including 21 males (87.5%), 3 females (12.5%). The age was (58.0±10.8) years (38-75 years). Pathology analysis showed ectopic lymphoid-like structures were located in 16 (66.7%) cases and Russell bodies were detected in infiltrative plasma cells of 19(79.2%) cases with IgG4-TIN. Compared with cases without Russell body formation, cases with Russell body formation in renal interstitial plasma cells were more prone to show ectopic germinal center-like structure formation (P=0.001), tubular basement membrane (TBM) electron dense deposits (P=0.040) and reduced blood C3 levels (P=0.028).@*Conclusions@#Abnormal tubulointerstitial infiltration of ectopic lymphoid-like structures and plasma cells with prominent Russell body exist in IgG4-TIN patients, which suggests the persistent activation of lymphocytes and plasma cells in renal interstitium may contribute to the pathogenesis of IgG4-TIN.
ABSTRACT
Objective To investigate the clinical significance and pathological features of lymphocytes and plasma cells infiltration and related ectopic lymphoid-like structures in IgG4-related tubulointerstitial nephritis (IgG4-TIN). Methods Complete data was collected from 24 patients with IgG4-TIN confirmed by pathology in the Peking University First Hospital. The renal specimens were examined by routine light microscopy, immunofluorescence and electron microscopy examination. In addition, immunohistochemistry was used to detect the distribution of CD20+ B lymphocytes, CD3+ T lymphocytes and CD138+ plasma cells. Results A total of 24 patients were enrolled in the study, including 21 males (87.5％), 3 females (12.5％). The age was (58.0 ± 10.8) years (38-75 years). Pathology analysis showed ectopic lymphoid-like structures were located in 16 (66.7％) cases and Russell bodies were detected in infiltrative plasma cells of 19(79.2％) cases with IgG4-TIN. Compared with cases without Russell body formation, cases with Russell body formation in renal interstitial plasma cells were more prone to show ectopic germinal center-like structure formation (P=0.001), tubular basement membrane (TBM) electron dense deposits (P=0.040) and reduced blood C3 levels (P=0.028). Conclusions Abnormal tubulointerstitial infiltration of ectopic lymphoid-like structures and plasma cells with prominent Russell body exist in IgG4-TIN patients, which suggests the persistent activation of lymphocytes and plasma cells in renal interstitium may contribute to the pathogenesis of IgG4-TIN.
ABSTRACT
A rare gastric mucosal lesion characterized by Russell body-containing plasma cell infiltration is termed as Russell body gastritis. This lesion is highly suggested to be correlated with Helicobacter pylori-induced chronic gastritis, and often misdiagnosed as mucosa-associated lymphoid tissue lymphoma, signet ring cell carcinoma, plasmacytoma, or xanthoma. However, Russell body gastritis is easily discriminated by its polyclonal immunoreaction to immunoglobulin light chains contrary to monoclonal immunoreaction of neoplastic disease. We report here a case of Russell body gastritis associated with H. pylori infection, which disappeared after H. pylori eradication.
Subject(s)
Carcinoma, Signet Ring Cell , Gastritis , Helicobacter pylori , Helicobacter , Immunoglobulin Light Chains , Lymphoma, B-Cell, Marginal Zone , Plasma Cells , Plasmacytoma , Stomach , XanthomatosisABSTRACT
A 64-year-old man was found to have a nodule in his right lung. He also complained of nausea and abdominal pain during the clinical course. Esophagogastroduodenoscopy revealed a duodenal ulcer associated with severe stenosis and a suspicion of malignancy. However, three subsequent biopsies revealed no evidence of malignancy. The fourth biopsy showed scattered large eosinophilic cells with an eccentric nucleus, leading to a diagnosis of Russell body duodenitis (RBD). RBD is an extremely rare disease, and little is known about its etiology and clinical course. The pathogenesis of RBD is discussed based on our experience with this case.
Subject(s)
Humans , Middle Aged , Abdominal Pain , Biopsy , Constriction, Pathologic , Diagnosis , Duodenal Ulcer , Duodenitis , Endoscopy, Digestive System , Eosinophils , Lung , Nausea , Rare DiseasesABSTRACT
Russell body gastritis is a very rare gastric inflammatory lesion, which presents as dense infiltration of plasma cells containing immunoglobulin structures of Russell body. The lesion is closely associated with Helicobacter pylori-induced chronic gastritis and mimics malignant tumors, such as marginal zone B-cell lymphoma of mucosa-associated lymphoid tissues and signet-ring cell carcinoma. We report a case of Russell body gastritis which regressed 6 months after eradication of H. pylori. Herein we described endoscopic features of Russell body gastritis and histological details of differential diagnosis.
Subject(s)
Diagnosis, Differential , Gastritis , Helicobacter , Helicobacter pylori , Immunoglobulins , Lymphoid Tissue , Lymphoma, B-Cell, Marginal Zone , Plasma CellsABSTRACT
Russell body gastritis (RBG), which can be associated with Helicobacter pylori (HP) infection, is a recently acknowledged lesion characterized by the presence of eosinophilic intracytoplasmic inclusions in plasmacytes. Herein the authors discuss the morphological aspects of a case of RBG in a male patient with clinical complaint of epigastric pain. Upper endoscopy revealed areas of erythema/edema in the antrum. As far as microscopy is concerned, a mononuclear inflammatory infiltrate was identified on the lamina propria, with plasma cells containing Schiff acid periodic (PAS) positive eosinophilic intracytoplasmic globules, which showed positive immunostaining for CD79a, CD138, and kappa/lambda light chains. HP was identified by Giemsa stains.
A gastrite com corpúsculos de Russell (RBG), que pode estar associada à infecção por Helicobacter pylori (HP), é uma lesão recentemente reconhecida que se caracteriza pela presença de inclusões eosinofílicas intracitoplasmáticas em plasmócitos. Neste relato, os autores discutem os aspectos morfológicos de um caso de RBG em um paciente masculino com queixa clínica de dor epigástrica. A endoscopia revelou áreas de eritema/edema no antro. À microscopia, um infiltrado inflamatório mononuclear foi identificado na lâmina própria, com plasmócitos exibindo glóbulos eosinofílicos intracitoplasmáticos ácido periódico de Schiff (PAS) positivos, que apresentaram imunoexpressão positiva para CD79a,CD138 e cadeias leves kappa/lambda. HP foi identificado na coloração de Giemsa.
ABSTRACT
Russell body gastritis was first defined in 1998, but not many cases have been reported since then. The exact causes and process of this condition are unknown yet; however, considering the reported cases, it has been highly suggested to have correlation with Helicobacter pylori infection. Russell body gastritis has a non-specific clinical presentation of gastritis such as gastric mucosal edema in the macroscopic view. It can be mistaken as xanthoma, signet ring cell carcinoma, or a malignant lymphoma including mucosa-associated lymphoid tissue lymphoma and plasmocytoma. Russell body gastritis features polyclonal immunoglobulin and is differentiated from Mott cancer, of which immune globulin has monoclonal aspect. Authors report here two cases of Russell body gastritis with examined endoscopic findings as well as a review of related literature on the association of all reported cases of Russell body gastritis with H. pylori infection.
Subject(s)
Carcinoma, Signet Ring Cell , Edema , Gastritis , Helicobacter , Helicobacter pylori , Immunoglobulins , Lymphoma , Lymphoma, B-Cell, Marginal Zone , Plasmacytoma , XanthomatosisABSTRACT
Russell body gastritis is a very rare disease with an uncertain cause. The disease is often misdiagnosed as xanthoma, signet ring cell carcinoma, MALT lymphoma and plasmacytoma. Russell body gastritis is characterized by the polyclonic nature of immunoglobulin and usually tests positive to the kappa and lambda light chains. It is different from a Mott cell tumor, which shows monoclonal nature of immunoglobulin. Until now, few cases have been reported and most were associated with a Helicobacter pylori infection. We encountered a case of Russell body gastritis associated with a Helicobacter pylori infection, which showed complete improvement after eradicating the Helicobacter pylori infection. We report this case with review of the relevant literature.