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.
Copyright © 2018. Published by Elsevier Inc.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Agammaglobulinemia / complications
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Agammaglobulinemia / immunology*
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Agammaglobulinemia / therapy
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Aged
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Anti-Infective Agents / therapeutic use
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B-Lymphocytes / cytology*
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B-Lymphocytes / immunology
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Bone Marrow*
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Female
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Humans
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Immunoglobulins / therapeutic use
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Immunoglobulins, Intravenous / therapeutic use
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Immunologic Deficiency Syndromes / immunology
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Immunologic Deficiency Syndromes / therapy
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Immunologic Factors / therapeutic use
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Lymphopenia / complications
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Lymphopenia / immunology*
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Lymphopenia / therapy
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Lymphopoiesis / immunology*
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Male
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Middle Aged
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Precursor Cells, B-Lymphoid / cytology*
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Precursor Cells, B-Lymphoid / immunology
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Syndrome
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Thymectomy
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Thymoma / complications
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Thymoma / immunology*
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Thymoma / therapy
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Thymus Neoplasms / complications
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Thymus Neoplasms / immunology*
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Thymus Neoplasms / therapy
Substances
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Anti-Infective Agents
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Immunoglobulins
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Immunoglobulins, Intravenous
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Immunologic Factors