IgG4 plasma cell myeloma: new insights into the pathogenesis of IgG4-related disease
| Autor: | Scott Ely, Ruben Niesvizky, David Jayabalan, Shivakumar Subramaniyam, Susan Mathew, Attilio Orazi, Alexander I Geyer, Julia T. Geyer |
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| Rok vydání: | 2014 |
| Předmět: |
Male
Pathology medicine.medical_specialty Biology Immunoglobulin G Pathology and Forensic Medicine Pathogenesis parasitic diseases Plasma Cell Myeloma medicine Humans skin and connective tissue diseases Fasciitis Multiple myeloma Aged Aged 80 and over integumentary system fungi Middle Aged medicine.disease medicine.anatomical_structure Monoclonal Immunology biology.protein Female IgG4-related disease Bone marrow Multiple Myeloma |
| Zdroj: | Modern Pathology. 27:375-381 |
| ISSN: | 0893-3952 |
| DOI: | 10.1038/modpathol.2013.159 |
| Popis: | IgG4-related disease is a newly described systemic fibroinflammatory process, characterized by increase in IgG4-positive plasma cells. Its pathogenesis, including the role of IgG4, remains poorly understood. Plasma cell myeloma is typically associated with a large monoclonal serum spike, which is frequently of IgG isotype. We sought to identify and characterize a subset of IgG4-secreting myeloma, as it may provide a biological model of disease with high serum levels of IgG4. Six out of 158 bone marrow biopsies (4%) from patients with IgG myeloma expressed IgG4. Four patients were men and two were women, with a mean age of 64 (range 53-87) years. Imaging showed fullness of pancreatic head (1), small non-metabolic lymphadenopathy (1), and bone lytic lesions (6). Two patients developed necrotizing fasciitis. All had elevated serum M-protein (mean 2.4, range 0.5-4.2 g/dl), and none had definite signs or symptoms of IgG4-related disease. Four myelomas had plasmablastic morphology. Four had kappa and two had lambda light chain expression. Three cases expressed CD56. Two patients had a complex karyotype. In conclusion, the frequency of IgG4 myeloma correlates with the normal distribution of IgG4 isoform. The patients with IgG4 myeloma appear to have a high rate of plasmablastic morphology and could be predisposed to necrotizing fasciitis. Despite high serum levels of IgG4, none had evidence of IgG4-related disease. These findings suggest that the increased number of IgG4-positive plasma cells is not the primary etiologic agent in IgG4-related disease. Elevated serum levels of IgG4 is not sufficient to produce the typical disease presentation and should not be considered diagnostic of IgG4-related disease. |
| Databáze: | OpenAIRE |
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