Neutropenia Associated with Common Variable Immunodeficiency

Common variable immunodeficiency (CVID) is used to describe patients with hypogammaglobulinemia of undetermined origin. The IgG level is low but IgM and IgA levels vary. In vitro response of B cells to pokeweed mitogen to produce immunoglobulin is impaired in patients whose B cells fail to differentiate into mature cells.

Clinical Manifestations

CVID usually becomes manifest in patients between 20 and 30 years of age and only occasionally in childhood. Most patients manifest recurrent infections of the sinopul-monary tract. They are also prone to develop noncaseating granulomas of the skin, gut, and other organs. These patients are predisposed to develop autoimmune disorders such as immune thrombocytopenic purpura (ITP), systemic lupus erythe-matosus (SLE), and thyroiditis.

Treatment

Neutropenia in CVID is explained on an autoimmune basis. Patients respond to G-CSF treatment, IV immunoglobulin, and antibiotics.

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