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- Title
Modern management of primary B-cell immunodeficiencies.
- Authors
Hoernes, Miriam; Seger, Reinhard; Reichenbach, Janine
- 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.
- Publication
Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology, 2011, Vol 22, Issue 8, p758
- ISSN
1399-3038
- Publication type
Journal Article
- DOI
10.1111/j.1399-3038.2011.01236.x