NDLI: Common Variable Immune Deficiency in Children—Clinical Characteristics Varies Depending on Defect in Peripheral B Cell Maturation

Content Provider	Springer Nature Link
Author	Piątosa, Barbara Pac, Małgorzata Siewiera, Katarzyna Pietrucha, Barbara Klaudel Dreszler, Maja Heropolitańska Pliszka, Edyta Wolska Kuśnierz, Beata Dmeńska, Hanna Gregorek, Hanna Sokolnicka, Irena Rękawek, Aneta Tkaczyk, Katarzyna Bernatowska, Ewa
Copyright Year	2013
Abstract	Common variable immune deficiency (CVID) is a heterogeneous disease associated with ineffective production of antibodies. It is usually diagnosed in adulthood, but a variable proportion of children develop CVID. Early identification of patients with potentially worse prognosis may help to avoid serious complications. The goal of this study was to associate the clinical phenotype of patients with early onset CVID with peripheral B-cell maturation profile. Four color flow cytometry was used to define distribution of peripheral B-cell subsets in 49 children with early-onset CVID. All clinical data were extracted from medical records. A proportion of patients demonstrated diminishing with time total B-lymphocytes pool, beyond physiological age-related changes. Irrespective from duration of the follow-up period the B-cell maturation profile in individual patients remained unchanged. We identified six different aberrant peripheral B cell maturation profiles associated with different clinical characteristics. Patients with an early B-cell maturation block earlier required replacement therapy and were at significantly greater risk of enteropathy, granuloma formation, cytopenia, and lymphoproliferation. B-cell maturation inhibited at the natural effector stage was associated with higher risk of autoimmune manifestations other than autoimmune cytopenia. Prevalence of male patients was observed among patients with B-cell maturation inhibited at naïve B-cell stage. In conclusion, the diagnostic process in patients with suspected early-onset CVID shall include routine analysis of peripheral B-cell maturation to provide surrogate markers identifying patients at greater risk of developing certain complications.
Starting Page	731
Ending Page	741
Page Count	11
File Format	PDF
ISSN	02719142
Journal	Journal of Clinical Immunology
Volume Number	33
Issue Number	4
e-ISSN	15732592
Language	English
Publisher	Springer US
Publisher Date	2013-02-07
Publisher Place	Boston
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Common variable immune deficiency flow cytometry B lymphocytes defective B-cell maturation Immunology Infectious Diseases Internal Medicine Medical Microbiology
Content Type	Text
Resource Type	Article
Subject	Immunology and Allergy Immunology

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