Perifollicular granuloma is a unique histologic feature and whether it is associated with immunoglobulin G4(IgG 4)-related disease is controversial. We report a case of a 38-year-old man who presented with worsening l...Perifollicular granuloma is a unique histologic feature and whether it is associated with immunoglobulin G4(IgG 4)-related disease is controversial. We report a case of a 38-year-old man who presented with worsening left eye pain, proptosis, tearing, gritty sensation, blurred vision and multiple lymphadenopathy. An axillary lymph node resection showed reactive follicular and interfollicular lymph node hyperplasia, and increased eosinophils and plasma cells(at least 80% of Ig G+ plasma cells were positive for IgG 4). A distinct feature was the presence of multifocal, perifollicular histiocytic granulomas, which formed a wreath around the entire follicles. The human herpes virus 8 was not detected by immunohistochemistry. In addition, an extensive panel of special stains, immunohistochemistry, and flow cytometry was negative for lymphoma, fungal, or mycobacterial infection. The findings were suggestive of IgG 4-related sclerosing disease-associated lymphadenopathy. Further laboratory testing showed a significant increase of serum immunoglobulin E(> 23000 IU/mL) and slight increase of total Ig G, but normal serum Ig G4. Even though perifollicular granuloma is a nonspecific histopathologic feature and can be seen in other diseases, such as nodular lymphocyte predominant Hodgkin lymphoma, Ig G4-related lymphadenopathy should be listed in the differential diagnoses of benign reactive lymph nodes, especially when perifollicular granuloma and plasmacytosis coexist.展开更多
Here we report the clinical,radiological and neuropathological findings of a patient with tumor-like inflammatory demyelinating diseases of the central nervous system.The patient was a 51-year-old man with a four-mont...Here we report the clinical,radiological and neuropathological findings of a patient with tumor-like inflammatory demyelinating diseases of the central nervous system.The patient was a 51-year-old man with a four-month history of inflammatory pseudotumor and no other significant medical history,who presented to our hospital recurrent relapse numbness and weakness of his right extremities,dysarthria and memory deterioration.Brain magnetic resonance imaging(MRI) showed mass focal lesion in white matter of left parietal lobes.The biopsy showed numerous infiltrating macrophages and lymphocytes within the perivascular.The patient responded clinically to corticosteroid and intravenous immunoglobulin(IVIG) therapy.According to the results of the biopsy and the MRI,a diagnosis of inflammatory pseu-dotumor of the central nervous system was made.The vascular dysfunction may act in the pathogenesis of inflammatory pseudotumor of the central nervous system.展开更多
基金Supported by Department of Pathology,the University of Texas Health Science Center at Houston,United States
文摘Perifollicular granuloma is a unique histologic feature and whether it is associated with immunoglobulin G4(IgG 4)-related disease is controversial. We report a case of a 38-year-old man who presented with worsening left eye pain, proptosis, tearing, gritty sensation, blurred vision and multiple lymphadenopathy. An axillary lymph node resection showed reactive follicular and interfollicular lymph node hyperplasia, and increased eosinophils and plasma cells(at least 80% of Ig G+ plasma cells were positive for IgG 4). A distinct feature was the presence of multifocal, perifollicular histiocytic granulomas, which formed a wreath around the entire follicles. The human herpes virus 8 was not detected by immunohistochemistry. In addition, an extensive panel of special stains, immunohistochemistry, and flow cytometry was negative for lymphoma, fungal, or mycobacterial infection. The findings were suggestive of IgG 4-related sclerosing disease-associated lymphadenopathy. Further laboratory testing showed a significant increase of serum immunoglobulin E(> 23000 IU/mL) and slight increase of total Ig G, but normal serum Ig G4. Even though perifollicular granuloma is a nonspecific histopathologic feature and can be seen in other diseases, such as nodular lymphocyte predominant Hodgkin lymphoma, Ig G4-related lymphadenopathy should be listed in the differential diagnoses of benign reactive lymph nodes, especially when perifollicular granuloma and plasmacytosis coexist.
文摘Here we report the clinical,radiological and neuropathological findings of a patient with tumor-like inflammatory demyelinating diseases of the central nervous system.The patient was a 51-year-old man with a four-month history of inflammatory pseudotumor and no other significant medical history,who presented to our hospital recurrent relapse numbness and weakness of his right extremities,dysarthria and memory deterioration.Brain magnetic resonance imaging(MRI) showed mass focal lesion in white matter of left parietal lobes.The biopsy showed numerous infiltrating macrophages and lymphocytes within the perivascular.The patient responded clinically to corticosteroid and intravenous immunoglobulin(IVIG) therapy.According to the results of the biopsy and the MRI,a diagnosis of inflammatory pseu-dotumor of the central nervous system was made.The vascular dysfunction may act in the pathogenesis of inflammatory pseudotumor of the central nervous system.
