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Precursor B-cell development in bone marrow of Good syndrome patients. 2019
Lucía Del Pino Molina, and
Marjolein Wentink, and
Marcel van Deuren, and
P Martin van Hagen, and
C I Edvard Smith, and
Mirjam van der Burg
Dept. of Clinical Immunology, La Paz University Hospital, Lymphocyte Pathophysiology in Immunodeficiencies Group La Paz Institute for Health Research (IdiPAZ), Madrid, Spain.
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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