Gammopathy and B lymphocyte clonality in patients with Gaucher type I disease |
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| Authors: | Predrag Rodic Sonja Pavlovic Tatjana Kostic Nada Suvajdzic Vukovic Maja Djordjevic Zorica Sumarac Marijana Dajak Branka Bonaci Nikolic Dragana Janic |
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| Affiliation: | 1. Department of Hematology and Oncology, University Children''s Hospital, Belgrade, Serbia;2. Institute of Molecular Genetics and Genetic Engineering, University of Belgrade, Belgrade, Serbia;3. Clinic of Hematology, Clinical Center of Serbia, Belgrade, Serbia;4. Department of Metabolic Diseases, Mother and Child Healthcare Institute, Belgrade, Serbia;5. Center of Medical Biochemistry, Clinical Center of Serbia, Belgrade, Serbia;6. Department of Allergy and Clinical Immunology, Clinical Center of Serbia, Belgrade, Serbia;7. Faculty of Medicine, University of Belgrade, Belgrade, Serbia |
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| Abstract: | ![]()
IntroductionWe evaluated a novel approach for investigation of lymphocyte dysregulation in Gaucher patients by including determination of IgH and TCR gene rearrangements together with levels of immunoglobulins, natural autoantibodies as well as presence of monoclonal protein.Materials and methodsMeasurement of serum immunoglobulins, monoclonal immunoglobulins, selected autoantibodies, as well as analysis of immunoglobulin heavy chain and T cell receptor gene rearrangements.ResultsImmunoglobulin disorder was detected in 29.6% patients, 40.7% demonstrated presence of B cell clonality and 44.4% demonstrated presence of autoantibodies. In five patients in our series, the presence of IgH gene rearrangement was the only detectable indicator of B cell dysfunction. TCR gene rearrangements were not found in any of the patients.ConclusionBased on our results, we propose IgH gene rearrangements as a new biomarker for investigation of B cell dysfunction occurring as a complication of Gaucher disease. |
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