The syndrome of cellular immunodeficiency with immunoglobulins

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

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51 Scopus citations

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 - Feb 1974

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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    Lawlor, G. J., Ammann, A. J., Wright, W. C., La Franchi, S. H., 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