B Cell Immunophenotyping in Common Variable Immunodeficiency (CVID) v1
Research type
Research Study
Full title
B Cell Immunophenotyping in Common Variable Immunodeficiency (CVID) age-related cohorts; does older age at presentation suggest a different disease process?
IRAS ID
173105
Contact name
Claire Bethune
Contact email
Sponsor organisation
Plymouth Hospitals NHS Trust (PHNT)
Duration of Study in the UK
1 years, 1 months, 28 days
Research summary
Common variable immune deficiencies (CVID) are a heterogeneous group of disorders where the immune system fails to produce protective antibodies, thereby leaving the body prone to persistent or repeated bacterial infections. These often affect the sinuses, chest, lungs or other places which come into direct contact with infectious agents.
The pathophysiology of CVID is poorly understood, and the diagnosis is one of exclusion. Treatment with intravenous (IV) or subcutaneous (SC) immunoglobulin replacement is used to reduce the number and severity of bacterial infections, however patients often acquire secondary complications which include bronchiectasis, autoimmunity, gastrointestinal (GI) problems, splenomegaly, and malignancies. Development of these complications is associated with a poor response to immunoglobulin therapy.
There have been several studies published to sub-classify CVID patients into distinct groups based on B cell immunophenotypes (characteristics) and clinical features. In clinical practice, the most widely accepted is EUROclass which accumulated data from eight countries across Europe in an attempt to consolidate previous findings. While this demonstrated individual characteristics associated with more complicated disease, it did not take age into account.
Clinical observation of patients with a diagnosis of CVID at Derriford Hospital suggests that there are two distinct cohorts: one group characterised by patients presenting in the third or fourth decade of life, and who have a more complicated disease course; and a second group characterised by presentation in older age (seventh or eighth decade of life) and with a less complicated disease course. CVID phenotypes are known to vary between adults and children, raising the possibility that the two groups of adults may also be pathophysiologically different.
This study aims to analyse B cell characteristics to identify naïve, non-switched memory, class switched memory, transitional, plasmablasts and IgM only memory cells to investigate the correlation between age at diagnosis clinical presentation and lymphocyte immunophenotype.REC name
East Midlands - Leicester South Research Ethics Committee
REC reference
15/EM/0100
Date of REC Opinion
19 Feb 2015
REC opinion
Favourable Opinion