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Clin Immunol Immunopathol. 1991 Nov;61(2 Pt 2):S94-9.

X-linked severe combined immunodeficiency.

Author information

1
Department of Pediatrics, University of Tennessee College of Medicine, Memphis.

Abstract

Between a third and half of all males with SCID and no family history of immunodeficiency represent the first manifestation in their family of a new mutation of the gene that causes X-linked SCID. These patients, like boys with a positive family history of X-linked SCID, have markedly reduced numbers of T cells, elevated numbers of B cells, and hypogammaglobulinemia. The hypogammaglobulinemia is due, at least in part, to the expression of the gene defect in B cells as well as in T cells. Patients with X-linked SCID who are treated with bone marrow transplant tend to engraft T cells readily but they do not engraft B cells unless they are treated with cytoreductive therapy prior to transplant. B-cell function after transplant tends to be poor, even in patients who have received transplants from HLA matched siblings. Better transplant strategies are required to achieve optimum long-term results in patients with X-linked SCID.

PMID:
1934618
DOI:
10.1016/s0090-1229(05)80043-1
[Indexed for MEDLINE]

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