Polyclonal hyperviscosity syndrome in IgG4-related disease and associated conditions
نویسندگان
چکیده
Polyclonal hyperviscosity syndrome (HVS) is rare and has been reported in various disorders of immune dysregulation and lymphoid hyperplasia. IgG4-Related Disease (IgG4-RD) is an emerging disorder often associated with exuberant hypergammaglobulinemia, and this review of seven cases establishes IgG4-RD as an important cause of polyclonal HVS.
منابع مشابه
[IgG4-related disease].
IgG4-related disease is a recently recognized systemic syndrome characterized by mass-forming lesions, mainly in exocrine tissue, that consist of lymphoplasmacytic infiltrates and sclerosis. There are numerous IgG4+ plasma cells in the affected tissues, and the serum IgG4 level is elevated in these patients. Ocular adnexal IgG4-related disease frequently involves bilateral lacrimal glands, and ...
متن کاملRelatively restricted migration of polyclonal IgG4 may mimic a monoclonal gammopathy in IgG4-related disease.
OBJECTIVES IgG4-related disease (IgG4-RD) is an increasingly recognized syndrome of unknown etiology that can affect a wide variety of organs. The commonly shared features include tumor-like swelling of the involved organs, a lymphoplasmacytic infiltrate enriched with polyclonal IgG4-positive plasma cells, variable degree of fibrosis, and elevated serum concentrations of polyclonal IgG4. METH...
متن کاملPolyclonal gammopathy with marked increase in serum viscosity.
A 45-year-old man with polyclonal hypergammaglobulinemia (gamma globulins, 102 g/L) had a serum relative viscosity of 13 nu but did not manifest clinical signs of hyperviscosity syndrome (e.g., retinopathy, bleeding diathesis, and neurological alterations), except for fatigue and anorexia. In contrast with other patients with polyclonal hyperviscosity reported so far, this patient did not have ...
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Statement of Purpose. IgG4-related disease (IgG4-RD) is usually associated to an increase of serum IgG4 levels. However other conditions have also been associated to high serum IgG4 levels. Methods. All IgG subclasses analyses performed in our hospital over a one-year period were analyzed. When IgG4 level were over 1.35 g/L, the patient's clinical observation was analyzed and both final diagnos...
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Proliferated IgG4(+) plasma cells are polyclonal, suggesting that the pathogenesis of IgG4-related disease (IgG4-RD) involves upstream events related to the regulation of IgG4 expansion. We hypothesized that lymphoid follicle formation may play an important role in the pathogenesis of IgG4-RD. Using various antibodies, especially against monocyte, macrophage, and follicular dendritic cell marke...
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