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Bone Marrow Clonogenic Capability, Cytokine Production, and Thymic Output in Patients with Common Variable Immunodeficiency¹

Antonella Isgrò^*, Marco Marziali^*, Ivano Mezzaroma^*, Giuseppe Luzi, Anna Maria Mazzone^*, Vanessa Guazzi^*, Grazia Andolfi, Barbara Cassani, Alessandro Aiuti and Fernando Aiuti^2,*

^* Interregional Center for Primary Immunodeficiencies, Department of Clinical Medicine, University of Rome "La Sapienza," and Azienda Policlinico Umberto I, and Division of Internal Medicine and Clinical Immunology, Second Faculty of Medicine, University of Rome "La Sapienza," Sant’Andrea Hospital, Rome, Italy; and San Raffaele Telethon Institute for Gene Therapy, Scientific Institute H. S. Raffaele, Milan, Italy

In patients with primary Ab deficiencies, hematological and immunological abnormalities are frequently observed. A regenerative failure of hemopoietic stem/progenitor cells has been hypothesized. We evaluated in the bone marrow (BM) of 11 patients with common variable immunodeficiency, the phenotype of BM progenitors and their in vitro growth by colony-forming cell (CFC) and long-term culture (LTC) assays. A significant decrease in erythroid and mixed CFC and, to a greater extent, in primitive LTC-CFC progenitors was observed in patients compared with healthy controls. The frequency of BM pre-B and pro-B cells correlated directly with the absolute number of CD19⁺ lymphocytes. BM cells cultured in vitro produced spontaneously lower amounts of IL-2 and elevated levels of TNF- compared with controls, indicating a skewing toward a proapoptotic cytokine pattern. In addition, stromal cells generated after BM LTC secreted less IL-7 and displayed by immunohistochemistry an altered phenotype. These findings were associated with a significant decrease in naive Th cells coexpressing CD31 in the peripheral blood. These results indicate an impaired growth and differentiation capacity of progenitor cells in patients with common variable immunodeficiency.

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