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• W1817156773 endingPage "410" @default.
• W1817156773 startingPage "381" @default.
• W1817156773 abstract "Publisher Summary Transplantation in SCID patients has represented the first clinical application of many of the advances that have occurred in clinical bone marrow transplantation during the last 20 years. SCID is not a single primary defect but is a clinical phenotype characterized by an absence of antigen-specific T and B lymphocyte immunity. At least a dozen different primary defects have been identified that can produce the SCID phenotype. As modern molecular genetic techniques improve, additional primary defects will be defined because at present the primary defect can be identified in less than 50% of SCID patients. Abnormalities that occur at either the pluripotent or lymphoid stem cell level will result in an absence of both circulating T and B lymphocytes. The majority of SCID patients has selective defects in T lymphocyte differentiation/function and has phenotypically and functionally normal B lymphocytes. Patients with the IL-2 deficient form of SCID have been difficult to transplant because of the fact that the patients' T lymphocytes are capable of normal proliferation and differentiation if an exogenous source of IL-2 is present. If the patients develop acute graft–versus-host disease following transplantation, phenotypically normal T lymphocytes of recipient origin can be detected. IL-2-deficient SCID patients can be successfully transplanted only if they are cytoreduced prior to transplantation to eliminate the circulating T lymphocytes and the abnormal stem cells." @default.
• W1817156773 created "2016-06-24" @default.
• W1817156773 creator A5045306382 @default.
• W1817156773 date "1991-01-01" @default.
• W1817156773 modified "2023-10-17" @default.
• W1817156773 title "The Biology of Bone Marrow Transplantation for Severe Combined Immune Deficiency" @default.
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