The syndrome of cellular immunodeficiency with immunoglobulins

Glenn J. Lawlor, Arthur J. Ammann, William C. Wright, Stephen Lafranchi, David Bilstrom, E. Richard Stiehm

Research output: Contribution to journalArticle

50 Citations (Scopus)

Abstract

The clinical and immunologic features of six patients with combined immunodeficiency with immunoglobulins are presented and are compared with 28 previously reported cases. Although the pathologic findings of these (thymic dysplasia with absence of Hassall's corpuscles and lymphoid hypoplasia and poor corticomedullarly differentiation) resemble those of severe combined immunodeficiency, there are sufficient features to characterize them as a distinct syndrome. Characteristic features are gradual onset, variable course, prolonged survival, lymphopenia with impaired cellular immunity, the presence of immunoglobulins and plasma cells, but with impaired antibody synthesis. We therefore feel that these cases can be recognized as a distinct primary immunodeficiency using the nomenclature cellular immunodeficiency with immunoglobulins.

Original languageEnglish (US)
Pages (from-to)183-192
Number of pages10
JournalThe Journal of Pediatrics
Volume84
Issue number2
DOIs
StatePublished - 1974
Externally publishedYes

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Immunoglobulins
Severe Combined Immunodeficiency
Lymphopenia
Plasma Cells
Terminology
Cellular Immunity
Survival
Antibodies

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Lawlor, G. J., Ammann, A. J., Wright, W. C., Lafranchi, S., Bilstrom, D., & Stiehm, E. R. (1974). The syndrome of cellular immunodeficiency with immunoglobulins. The Journal of Pediatrics, 84(2), 183-192. https://doi.org/10.1016/S0022-3476(74)80599-8

The syndrome of cellular immunodeficiency with immunoglobulins. / Lawlor, Glenn J.; Ammann, Arthur J.; Wright, William C.; Lafranchi, Stephen; Bilstrom, David; Stiehm, E. Richard.

In: The Journal of Pediatrics, Vol. 84, No. 2, 1974, p. 183-192.

Research output: Contribution to journalArticle

Lawlor, GJ, Ammann, AJ, Wright, WC, Lafranchi, S, Bilstrom, D & Stiehm, ER 1974, 'The syndrome of cellular immunodeficiency with immunoglobulins', The Journal of Pediatrics, vol. 84, no. 2, pp. 183-192. https://doi.org/10.1016/S0022-3476(74)80599-8
Lawlor GJ, Ammann AJ, Wright WC, Lafranchi S, Bilstrom D, Stiehm ER. The syndrome of cellular immunodeficiency with immunoglobulins. The Journal of Pediatrics. 1974;84(2):183-192. https://doi.org/10.1016/S0022-3476(74)80599-8
Lawlor, Glenn J. ; Ammann, Arthur J. ; Wright, William C. ; Lafranchi, Stephen ; Bilstrom, David ; Stiehm, E. Richard. / The syndrome of cellular immunodeficiency with immunoglobulins. In: The Journal of Pediatrics. 1974 ; Vol. 84, No. 2. pp. 183-192.
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