Forschungs- & Entwicklungsinformationsdienst der Gemeinschaft - CORDIS

A rapid, standardised classification of CVID

Primary antibody deficiencies (PAD) are rare diseases caused by defects of the immune systerm. These defects result in an individual having problems fighting bacterial infections so that they suffer recurrent sinusitis, bronchitis or even pneumonia. This usually leads to permanent lung damage and other life-threatening situations.

Early diagnosis and better classification is essential to prevent disability. We have developed an improved rapid and reliable classification of CVID the most common form of PAD. It is based on B cell subset analysis taking into account a direct relationship between numbers of class-switched memory B cells and immunoglobulin synthesis in vitro. Thus 75% of all CVID patients (type I) lack class-switched memory B cells (easily measurable by cytofluorometry) in their peripheral blood and fail to produce IgG and IgA in vivo and in vitro. 25% of CVID patients (type II) have low serum IgG and IgA concentrations but do have class-switched memory B cells and produce switched isotypes (IgG, IgA) in vitro. Attempts have been made to subclassify type I CVID patients based on the observation that a subset of patients with splenomegaly and autoimmune phenomena often exhibits an expansion of CD21neg B cells. The group of E.Oksenhendler in Paris has identified among type I CVID patients a subset which lacks IgM memory B cells as well as class-switched memory B cells.Currently a European consensus study of >300 CVID patients is being finalized confirming basically the Freiburg classification but extending it in subclassifying type I patients.
This European consensus classification of CVID will considerably improve the speed of diagnosis and treatment decisions for CVID patients throughout the world.

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