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 language | English (US) |
|---|---|
| Pages (from-to) | 183-192 |
| Number of pages | 10 |
| Journal | The Journal of Pediatrics |
| Volume | 84 |
| Issue number | 2 |
| DOIs | |
| State | Published - 1974 |
| Externally published | Yes |
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ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
Cite this
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 journal › Article
}
TY - JOUR
T1 - The syndrome of cellular immunodeficiency with immunoglobulins
AU - Lawlor, Glenn J.
AU - Ammann, Arthur J.
AU - Wright, William C.
AU - Lafranchi, Stephen
AU - Bilstrom, David
AU - Stiehm, E. Richard
PY - 1974
Y1 - 1974
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=0015994130&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0015994130&partnerID=8YFLogxK
U2 - 10.1016/S0022-3476(74)80599-8
DO - 10.1016/S0022-3476(74)80599-8
M3 - Article
C2 - 4810725
AN - SCOPUS:0015994130
VL - 84
SP - 183
EP - 192
JO - Journal of Pediatrics
JF - Journal of Pediatrics
SN - 0022-3476
IS - 2
ER -