Immunoglobulin and free light chain abnormalities in Gaucher disease type I: data from an adult cohort of 63 patients and review of the literature |
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Authors: | M de Fost T A Out F A de Wilde E P M Tjin S T Pals M H J van Oers R G Boot J F M G Aerts M Maas S vom Dahl C E M Hollak |
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Institution: | Department of Endocrinology and Metabolism, F4-279, University of Amsterdam, Academic Medical Center, P.O. Box 22700, 1100 DD Amsterdam, The Netherlands. |
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Abstract: | Gaucher disease type I, the most common lysosomal storage disorder, is associated with immunoglobulin abnormalities. We studied
the prevalence, risk factors, pathogenesis, and effect of enzyme relation therapy (ERT) on gammopathies in an adult Gaucher
disease type I cohort (N = 63) and related the results to a review of the currently available literature. Polyclonal gammopathies and monoclonal gammopathy
of undetermined significance (MGUS) in our adult GD I cohort were found in 41% and 19% of patients. These results are similar
to the data from the literature and correspond to the increased risk of multiple myeloma (MM) that has been described. The
prevalence of MGUS in our cohort increased with age but was not associated with disease severity or exposure time. The serum
levels of free light chains of immunoglobulins were measured and were not found predictive for the development of MGUS or
MM. Levels of pro- as well as anti-inflammatory cytokines, growth factors, and chemokines, especially those involved in inflammation
and B-cell function, are disturbed in GD I, with the most impressive and consisting elevations for interleukin-10 and pulmonary
and activation-regulated chemokine. A beneficial effect of ERT on the occurrence and progression of gammopathies was suggested
from longitudinal data. |
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Keywords: | Gaucher disease MGUS Multiple myeloma Free light chains Cytokines |
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