Article Sections

Introduction

Common variable immunodeficiency (CVID) is a primary immunodeficiency disorder characterized by impaired differentiation of mature B cells into plasma cells.  Patients present in adolescence or early adulthood with recurrent respiratory and gastrointestinal infections due to hypogammaglobulinemia, and life-long immunoglobulin replacement therapy is required.

Pathogenesis

Normal B cell development begins in the bone marrow, where hematopoietic stem cells differentiate into immature B cells.  Immature B cells migrate to peripheral lymphoid tissue (eg, spleen, lymph nodes) ( Figure 1).  Upon exposure to an antigen, immature B cells undergo activation, proliferation, and differentiation into plasma cells, as follows:

  • In the absence of T cells, most mature B cells differentiate into short-lived plasma cells that produce antigen-specific IgM antibodies.
  • In the presence of T cells, mature B cells undergo isotype switching (ie, immunoglobulin class switching (

Continue Learning with UWorld

Get the full Common Variable Immunodeficiency (CVID) article plus rich visuals, real-world cases, and in-depth insights from medical experts, all available through the UWorld Medical Library.

Figures

Figure 1
Figure 1

Tables

Table 1