Chemotactic defects in severe combined immunodeficiency

Cellular and humoral components of leukotaxis were studied serially in four male infants with severe combined immunodeficiency disease. Two of the four, both lacking B and T cells initially, had a significant defect in neutrophil and monocyte chemotaxis. The other two, who had a high number of immun...

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Published inThe Journal of pediatrics Vol. 92; no. 1; pp. 43 - 50
Main Authors Pahwa, Savita G., Smithwick, Elizabeth M., Grimes, Elena R., O'Reilly, Richard J., Pahwa, Rajendra N., Good, Robert A.
Format Journal Article
LanguageEnglish
Published United States Mosby, Inc 1978
Subjects
B-Lymphocytes - immunology
B-Lymphocytes - physiology
Bone Marrow Transplantation
Chemotaxis, Leukocyte
Complement System Proteins - metabolism
Endotoxins
Humans
Immunologic Deficiency Syndromes - immunology
Infant
Infant, Newborn
Male
Monocytes - immunology
Monocytes - physiology
Neutrophils - immunology
Neutrophils - physiology
T-Lymphocytes - immunology
T-Lymphocytes - physiology
Transplantation, Autologous
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Summary:Cellular and humoral components of leukotaxis were studied serially in four male infants with severe combined immunodeficiency disease. Two of the four, both lacking B and T cells initially, had a significant defect in neutrophil and monocyte chemotaxis. The other two, who had a high number of immunoglobulin-bearing cells (B cells), did not have these cellular abnormalities. In contrast, defective generation of chemotactic factor following endotoxin activation was observed in all patients. The defects were corrected coincident with or soon after successful engraftment of either bone marrow or fetal tissues. The reported deficiencies may be another manifestation of the heterogeneity in SCID.
Bibliography:ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
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ISSN:0022-3476
1097-6833
DOI:10.1016/S0022-3476(78)80068-7