CVID represents a heterogeneous immunodeficiency syndrome characterized by hypogammaglobulinemia and recurrent sinopulmonary infections. We have encountered 3 CVID patients with profound longstanding hypogammaglobulinemia whose histories are conspicuous for the absence of recurrent infections. This paradox suggests that these patients are protected by a non-immunoglobulin dependent host defense mechanism.

The clinical histories of 3 patients (2 males and 1 female) with CVID and no history of recurrent infections evaluated at our institution were reviewed.

Documentation of hypogammaglobulinemia in each patient was made between 6 and 14 years ago. The diagnosis of CVID was made between ages 25 and 46. Each denied a history of pneumonia, otitis, or recurrent sinusitis. Serum IgG ranged from 57-169 mg/dl (650-2000 mg/dl), IgM ranged from 10-25 mg/dl (40-270 mg/dl) and IgA was <8 mg/dl in each patient (50-100 mg/dl). Serum albumin ranged from 4.1-4.5 g/dl. None of the patients mounted protective antibody titers when vaccinated with Pneumovax. Flow cytometry revealed normal numbers of CD19+ B cells.

We describe a subset of CVID patients who were spared recurrent sinopulmonary infections over a period of years, despite quantitative and functional immunoglobulin dysfunction. Study of these patients may provide insights into novel anti-bacterial host defense mechanisms that can offset the infectious complications of hypogammaglobulinemia.