
Severe combined immunodeficiency with leukopenia (reticular dysgenesis) in siblings: immunologic and histopathologic findings.
Publication
, Journal Article
Ownby, DR; Pizzo, S; Blackmon, L; Gall, SA; Buckley, RH
Published in: J Pediatr
September 1976
The hematologic and histologic features of two, nontwin, male siblings with severe combined immunodeficiency and variable granulocytopenia are compared to the four previously reported cases of reticular dysgenesis. These sibs died at 50 and 3 days of age, respectively, with Pseudomonas sepsis and congenital cytomegalovirus infection, respectively. A maternal uncle has selective IgA deficiency. Cord blood from the second sib contained a normal percentage of E-rosetting lymphocytes; however, these lymphocytes failed to respond to mitogenic stimulation in vitro. Erythrocyte and lymphocyte levels of adenosine deaminase were elevated in the father and the second sib. Serum immunoglobulin concentrations were low in both siblings.
Duke Scholars
Published In
J Pediatr
DOI
ISSN
0022-3476
Publication Date
September 1976
Volume
89
Issue
3
Start / End Page
382 / 387
Location
United States
Related Subject Headings
- Pediatrics
- Male
- Lymphocytes
- Leukopenia
- Infant, Newborn
- Infant
- Immunologic Deficiency Syndromes
- Immunoglobulin A
- Immune Adherence Reaction
- Humans
Citation
APA
Chicago
ICMJE
MLA
NLM
Ownby, D. R., Pizzo, S., Blackmon, L., Gall, S. A., & Buckley, R. H. (1976). Severe combined immunodeficiency with leukopenia (reticular dysgenesis) in siblings: immunologic and histopathologic findings. J Pediatr, 89(3), 382–387. https://doi.org/10.1016/s0022-3476(76)80532-x
Ownby, D. R., S. Pizzo, L. Blackmon, S. A. Gall, and R. H. Buckley. “Severe combined immunodeficiency with leukopenia (reticular dysgenesis) in siblings: immunologic and histopathologic findings.” J Pediatr 89, no. 3 (September 1976): 382–87. https://doi.org/10.1016/s0022-3476(76)80532-x.
Ownby DR, Pizzo S, Blackmon L, Gall SA, Buckley RH. Severe combined immunodeficiency with leukopenia (reticular dysgenesis) in siblings: immunologic and histopathologic findings. J Pediatr. 1976 Sep;89(3):382–7.
Ownby, D. R., et al. “Severe combined immunodeficiency with leukopenia (reticular dysgenesis) in siblings: immunologic and histopathologic findings.” J Pediatr, vol. 89, no. 3, Sept. 1976, pp. 382–87. Pubmed, doi:10.1016/s0022-3476(76)80532-x.
Ownby DR, Pizzo S, Blackmon L, Gall SA, Buckley RH. Severe combined immunodeficiency with leukopenia (reticular dysgenesis) in siblings: immunologic and histopathologic findings. J Pediatr. 1976 Sep;89(3):382–387.

Published In
J Pediatr
DOI
ISSN
0022-3476
Publication Date
September 1976
Volume
89
Issue
3
Start / End Page
382 / 387
Location
United States
Related Subject Headings
- Pediatrics
- Male
- Lymphocytes
- Leukopenia
- Infant, Newborn
- Infant
- Immunologic Deficiency Syndromes
- Immunoglobulin A
- Immune Adherence Reaction
- Humans