Modern management of primary B-cell immunodeficiencies

• Published in: Pediatric allergy and immunology Vol. 22; no. 8; pp. 758 - 769
• Main Authors: Hoernes, Miriam, Seger, Reinhard, Reichenbach, Janine
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01.12.2011; Blackwell; Wiley Subscription Services, Inc
• Subjects: agammaglobulinaemia; Animals; Antibiotics; Antigens, CD19 - genetics; Antimicrobial agents; B cell immunodeficiencies; B-Lymphocytes - immunology; B-Lymphocytes - metabolism; Biological and medical sciences; Bronchiectasis; class switch recombination defects; Class switching; Clinical trials; Common variable immunodeficiency; Defects; Drug therapy; Fundamental and applied biological sciences. Psychology; Fundamental immunology; General aspects; Hematopoietic Stem Cell Transplantation; Humans; hyper IgM syndromes; Hypersensitivity; Immunodeficiencies; Immunodeficiencies. Immunoglobulinopathies; Immunoglobulin Class Switching - genetics; Immunoglobulin G; immunoglobulin substitution; Immunoglobulins - biosynthesis; Immunoglobulins - genetics; Immunoglobulins - immunology; Immunoglobulins, Intravenous - therapeutic use; Immunologic Deficiency Syndromes - genetics; Immunologic Deficiency Syndromes - immunology; Immunologic Deficiency Syndromes - physiopathology; Immunologic Deficiency Syndromes - therapy; Immunopathology; Immunosuppressive agents; Intravenous administration; IVIg; Lymphocytes B; Medical sciences; Modern Management B cell defects; Recombination; Recurrent infection; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; SCIg; stem cell transplantation; Transplants & implants; Immunopathology; Pediatrics; Immunoglobulins; Recombination; Common variable immunodeficiency; Intravenous administration; hyper IgM syndromes; Agammaglobulinemia; immunoglobulin substitution; Hyperimmunoglobulinemia M syndrome; SCIg; IVIg; agammaglobulinaemia; B-Lymphocyte; Genetic disease; B cell immunodeficiencies; Modern Management B cell defects; Clinical management; Immunology; Immunoglobulinopathy; Substitution; Isotype switch; Primary; class switch recombination defects
• ISSN: 0905-6157; 1399-3038; 1399-3038
• DOI: 10.1111/j.1399-3038.2011.01236.x

To cite this article: Hoernes M, Seger R, Reichenbach J. Modern management of primary B‐cell immunodeficiencies. Pediatr Allergy Immunology 2011: 22: 758–769. B‐cell defects constitute the majority of primary immunodeficiencies. Although a heterogeneous group of diseases, all are characterized by the reduction in or absence of immunoglobulins and/or specific antimicrobial antibodies. Substitution of immunoglobulin G (IgG) is therefore the mainstay of treatment. While from the late 1970s, the intravenous route of administration was the most common, in the past decades, subcutaneous immunoglobulin replacement therapy has become more popular among patients and physicians. Independently of the optimal route of administration, dosage and IgG trough level remain subjects of debate. Higher IgG trough levels seem to improve the protection against recurrent infections and thus better prevent complications such as bronchiectasis. Some patients, however, achieve protection with IgG trough levels on the lower IgG limit of healthy persons. Therefore, an individual protective IgG trough level needs to be defined for each patient. Use of additional prophylactic antibiotics and immunosuppressive drugs differs amongst specialized immunodeficiency centres and clearly requires future investigation in multi‐centre trials. Haematopoietic stem cell transplantation (HSCT) is to date indicated as curative treatment in certain patients with B‐cell defects associated with cell deficiencies, for example in two class‐switch recombination defects and in selected severe forms of common variable immunodeficiency.