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Differential Diagnosis in Hypogammaglobulinemia

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Book cover Humoral Primary Immunodeficiencies

Part of the book series: Rare Diseases of the Immune System ((RDIS))

Abstract

Hypogammaglobulinemia is defined as an impaired production of antibodies. This condition represents a diagnostic challenge for clinicians, due to its association with many pathological entities with different manifestations and outcomes. Primary hypogammaglobulinemia is caused by defects in B-cell number and maturation, reduced survival and activation of B cells, and faulty interaction between B and T cells and is commonly observed in many primary immunodeficiencies. However, hypogammaglobulinemia secondary to cancer, chemotherapy, immunosuppressive therapy, chromosomal alterations, and protein-losing diseases is a widely recognized condition. The clinical relevance of secondary hypogammaglobulinemias has been increasingly recognized. Differential diagnosis could be puzzling because of similar clinical aspects in primary and secondary forms of hypogammaglobulinemias. Thus, for all patients with a low immunoglobulin serum level at the initial evaluation, defined causes of secondary hypogammaglobulinemias should be extensively ruled out. In particular, B-cell malignancies (mainly chronic lymphocytic leukemia) and plasma cell dyscrasias should be excluded in patients with low immunoglobulin serum levels.

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  1. Department of Molecular Medicine, Sapienza University of Rome, Rome, Italy

    Isabella Quinti, Cinzia Milito, Rossella Carello & Federica Pulvirenti

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  1. Isabella Quinti
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  2. Cinzia Milito
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  1. Careggi Hospital, Department of Clinical and Experimental Medicine, University of Florence, Firenze, Italy

    Mario Milco D'Elios

  2. University Medical Centre Freiburg, Department of Rheumatology and Clinical Immunology and Center for Chronic Immunodeficiency, Freiburg, Germany

    Marta Rizzi

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Quinti, I., Milito, C., Carello, R., Pulvirenti, F. (2019). Differential Diagnosis in Hypogammaglobulinemia. In: D'Elios, M., Rizzi, M. (eds) Humoral Primary Immunodeficiencies. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-319-91785-6_19

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