Bone marrow clonogenic capability, cytokine production, and thymic output in patients with common variable immunodeficiency
| Autor: | Ivano Mezzaroma, Fernando Aiuti, Marco Marziali, Barbara Cassani, Anna Maria Mazzone, Vanessa Guazzi, Alessandro Aiuti, Grazia Andolfi, Giuseppe Luzi, Antonella Isgrò |
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| Přispěvatelé: | Isgro', A, Marziali, M, Mezzaroma, I, Luzi, G, Mazzone, Am, Guazzi, V, Andolfi, G, Cassani, B, Aiuti, Alessandro, Aiuti, F. |
| Jazyk: | angličtina |
| Rok vydání: | 2005 |
| Předmět: |
Adult
Male Stromal cell medicine.medical_treatment Immunology Bone Marrow Cells Thymus Gland Biology In Vitro Techniques CD19 Colony-Forming Units Assay medicine Immunology and Allergy Humans Progenitor cell Clonogenic assay B-Lymphocytes Tumor Necrosis Factor-alpha Common variable immunodeficiency Interleukin-7 Middle Aged medicine.disease Hematopoietic Stem Cells Hematopoiesis Haematopoiesis Cytokine medicine.anatomical_structure Common Variable Immunodeficiency Case-Control Studies biology.protein Cytokines Interleukin-2 Female Bone marrow |
| Zdroj: | Scopus-Elsevier |
| Popis: | 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-α 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. |
| Databáze: | OpenAIRE |
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