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Modern management of primary B-cell immunodeficiencies


Hoernes, M; Seger, R; Reichenbach, J (2011). Modern management of primary B-cell immunodeficiencies. Pediatric Allergy and Immunology, 22(8):758-769.

Abstract

To cite this article: Hoernes M, Seger R, Reichenbach J. Modern management of primary B-cell immunodeficiencies. Pediatr Allergy Immunology 2011: 22: 758-769. ABSTRACT: 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.

Abstract

To cite this article: Hoernes M, Seger R, Reichenbach J. Modern management of primary B-cell immunodeficiencies. Pediatr Allergy Immunology 2011: 22: 758-769. ABSTRACT: 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.

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Additional indexing

Item Type:Journal Article, refereed, further contribution
Communities & Collections:04 Faculty of Medicine > University Children's Hospital Zurich > Medical Clinic
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:2011
Deposited On:19 Dec 2011 12:13
Last Modified:05 Apr 2016 15:13
Publisher:Wiley-Blackwell
ISSN:0905-6157
Publisher DOI:https://doi.org/10.1111/j.1399-3038.2011.01236.x
PubMed ID:22122788

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