Background: Hypogammaglobulinemia may be part of several different immunological or malignant conditions,\r\nand its origin is not always obvious. Furthermore, although autoimmune cytopenias are known to be associated\r\nwith common variable immunodeficiency (CVID) and even may precede signs of immunodeficiency, this is not\r\nalways recognized. Despite novel insight into the molecular immunology of common variable immunodeficiency,\r\nseveral areas of uncertainty remain. In addition, the full spectrum of immunological effects of the B cell depleting\r\nanti-CD20 antibody Rituximab has not been fully explored. To our knowledge this is the first report of development\r\nof CVID in a patient with normal immunoglobulin prior to Rituximab treatment.\r\nCase presentation: Here we describe the highly unusual clinical presentation of a 34-year old Caucasian male with\r\ntreatment refractory immune thrombocytopenic purpura and persistent lymphadenopathy, who was\r\nsplenectomized and received multiple courses of high-dose corticosteroid before treatment with Rituximab resulted\r\nin a sustained response. However, in the setting of severe pneumococcal meningitis, hypogammaglobulinemia was\r\ndiagnosed. An extensive immunological investigation was performed in order to characterize his immune status,\r\nand to distinguish between a primary immunodeficiency and a side effect of Rituximab treatment. We provide an\r\nextensive presentation and discussion of the literature on the basic immunology of CVID, the mechanism of action\r\nof Rituximab, and the immunopathogenesis of hypogammaglobulinemia observed in this patient.\r\nConclusions: We suggest that CVID should be ruled out in any patient with immune cytopenias in order to avoid\r\ndiagnostic delay. Likewise, we stress the importance of monitoring immunoglobulin levels before, during, and after\r\nRituximab therapy to identify patients with hypogammaglobulinemia to ensure initiation of immunoglobulin\r\nreplacement therapy in order to avoid life-threatening invasive bacterial infections. Recent reports indicate that\r\nRituximab is not contra-indicated for the treatment of CVID-associated thrombocytopenia, however concomitant\r\nimmunoglobulin substitution therapy is of fundamental importance to minimize the risk of infections. Therefore,\r\nlessons can be learned from this case report by clinicians caring for patients with immunodeficiencies,\r\nhaematological diseases or other autoimmune disorders, particularly, when Rituximab treatment may\r\nbe considered.
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