Postnatal development of T lymphocytes in a novel X-linked immunodeficiency disease

Frank C. Schmalstieg; Daniel P. Wirt; Linda T. Adkins; Edward G. Brooks; Susan D. Stansberry; Leonard E. Swischuk; Armond S. Goldman

doi:10.1016/0090-1229(92)90061-R

Postnatal development of T lymphocytes in a novel X-linked immunodeficiency disease

Frank C. Schmalstieg, Daniel P. Wirt, Linda T. Adkins, Edward G. Brooks, Susan D. Stansberry, Leonard E. Swischuk, Armond S. Goldman

Research output: Contribution to journal › Article › peer-review

10 Scopus citations

Abstract

We previously reported an X-linked combined immunode-ficiency disease (CID) characterized by immune deficiencies and complicating infections that were more moderate than those found in severe CID (SCID). Since other unstudied males in the family died in infancy, we questioned whether this T cell defect was more profound in early life. Subsequently, the development of blood T cells in an affected new-born male was examined. T cells were virtually undetectable at 48 hr. Over the next several months, CD4⁺ T cells (principally CD45RO⁺) rose to levels similar to those found in older affected males, but CD8⁺ T cells developed more slowly and never attained levels found in other affected males. Thus, this disease in early life mimics SCID and may pose a higher risk of fatal infections to affected individuals during that period. Finally, we speculate that the genetic defect may disrupt intrathymic development or selection of T cells.

Original language	English (US)
Pages (from-to)	71-77
Number of pages	7
Journal	Clinical Immunology and Immunopathology
Volume	64
Issue number	1
DOIs	https://doi.org/10.1016/0090-1229(92)90061-R
State	Published - Jul 1992
Externally published	Yes

ASJC Scopus subject areas

Immunology and Allergy
Pathology and Forensic Medicine
Immunology

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title = "Postnatal development of T lymphocytes in a novel X-linked immunodeficiency disease",

abstract = "We previously reported an X-linked combined immunode-ficiency disease (CID) characterized by immune deficiencies and complicating infections that were more moderate than those found in severe CID (SCID). Since other unstudied males in the family died in infancy, we questioned whether this T cell defect was more profound in early life. Subsequently, the development of blood T cells in an affected new-born male was examined. T cells were virtually undetectable at 48 hr. Over the next several months, CD4+ T cells (principally CD45RO+) rose to levels similar to those found in older affected males, but CD8+ T cells developed more slowly and never attained levels found in other affected males. Thus, this disease in early life mimics SCID and may pose a higher risk of fatal infections to affected individuals during that period. Finally, we speculate that the genetic defect may disrupt intrathymic development or selection of T cells.",

author = "Schmalstieg, {Frank C.} and Wirt, {Daniel P.} and Adkins, {Linda T.} and Brooks, {Edward G.} and Stansberry, {Susan D.} and Swischuk, {Leonard E.} and Goldman, {Armond S.}",

note = "Funding Information: 1 This work was presented to the Society for Pediatric Research and the American Pediatric Society on May 9, 1990, and was supported by the John Sealy Memorial Fund, the James W. McLaughlin Fellowship Fund, and Sandoz Pharmaceutics. 2 Abbreviations used: CID, combined immunodeficiency disease; SCID, severe combined immunodeficiency disease; CD, leukocyte cluster of differentiation; TCR, T cell antigen receptors.",

year = "1992",

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doi = "10.1016/0090-1229(92)90061-R",

language = "English (US)",

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AU - Schmalstieg, Frank C.

AU - Wirt, Daniel P.

AU - Adkins, Linda T.

AU - Brooks, Edward G.

AU - Stansberry, Susan D.

AU - Swischuk, Leonard E.

AU - Goldman, Armond S.

N1 - Funding Information: 1 This work was presented to the Society for Pediatric Research and the American Pediatric Society on May 9, 1990, and was supported by the John Sealy Memorial Fund, the James W. McLaughlin Fellowship Fund, and Sandoz Pharmaceutics. 2 Abbreviations used: CID, combined immunodeficiency disease; SCID, severe combined immunodeficiency disease; CD, leukocyte cluster of differentiation; TCR, T cell antigen receptors.

PY - 1992/7

Y1 - 1992/7

N2 - We previously reported an X-linked combined immunode-ficiency disease (CID) characterized by immune deficiencies and complicating infections that were more moderate than those found in severe CID (SCID). Since other unstudied males in the family died in infancy, we questioned whether this T cell defect was more profound in early life. Subsequently, the development of blood T cells in an affected new-born male was examined. T cells were virtually undetectable at 48 hr. Over the next several months, CD4+ T cells (principally CD45RO+) rose to levels similar to those found in older affected males, but CD8+ T cells developed more slowly and never attained levels found in other affected males. Thus, this disease in early life mimics SCID and may pose a higher risk of fatal infections to affected individuals during that period. Finally, we speculate that the genetic defect may disrupt intrathymic development or selection of T cells.

AB - We previously reported an X-linked combined immunode-ficiency disease (CID) characterized by immune deficiencies and complicating infections that were more moderate than those found in severe CID (SCID). Since other unstudied males in the family died in infancy, we questioned whether this T cell defect was more profound in early life. Subsequently, the development of blood T cells in an affected new-born male was examined. T cells were virtually undetectable at 48 hr. Over the next several months, CD4+ T cells (principally CD45RO+) rose to levels similar to those found in older affected males, but CD8+ T cells developed more slowly and never attained levels found in other affected males. Thus, this disease in early life mimics SCID and may pose a higher risk of fatal infections to affected individuals during that period. Finally, we speculate that the genetic defect may disrupt intrathymic development or selection of T cells.

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Postnatal development of T lymphocytes in a novel X-linked immunodeficiency disease

Abstract

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