Publication date: Available online 25 January 2019
Source: Clinical Immunology
Author(s): Lucía del Pino Molina, Marjolein Wentink, Marcel van Deuren, Martin van Hagen, C.I. Edvard Smith, Mirjam van der Burg
Abstract
Good syndrome is an immunodeficiency presenting with thymoma, hypogammaglobulinemia and almost absent B cells. To investigate the origin of the B-cell lymphopenia in these patients, we studied B cell differentiation in the bone marrow of Good syndrome patients. We found very low numbers of precursor B cells in bone marrow of Good syndrome patients and a differentiation arrest after the pro-B-cell stage; this is different from other agammaglobulinemia patients with a defect in pre B-cell receptor signaling.
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