Abstract
The treatment of primary immunodeficiencies (PID) includes curative therapy and supportive therapy. As the curative therapy, stem cell transplantations (SCT), including BMT, PBSCT, and CBSCT, have been performed for severe combined immunodeficiencies (SCID), Wiskott-Aldrich syndrome, X-linked hyper-IgM syndrome, and other primary immunodeficiency diseases. Gene therapy is another curative therapy. X-SCID has recently been successfully treated by transducing common gamma chain into the patients' CD34 positive hematopoietic cells by using retroviral vector. Intravenous immunoglobulin infusion is the effective supportive therapy for patients with hypogammaglobulinemia. Patients with T cell defects are susceptible for P. carinii, Cryptosporidium pavum, fungs, and virus, and prevention by using antimicrobes must be considered. Ex vivo activated T cell infusion is a new immunotherapy, and our preliminary results have revealed promising effects for the management of infections in PID patients. In summary, an early diagnosis with early prevention of infections and early SCT if applicable is recommended as a strategy for the treatment of PID.
