Abstract
EBV-infected cell lines from 31 immunodeficient (ID) patients and 1 normal individual, and 7 spontaneous cell lines from cancer patients were assayed for terminal deoxynucleotidyl transferase (TdT) activity. TdT is found primarily in pre-T cells in bone marrow, thymus, and in some acute leukemias; it is not present in mature circulating B or T cells. Twenty-nine lines were studied with and without ATP, a specific TdT inhibitor, and 11 lines without ATP. As a reference, 8402 cells (T cell line) had 0.400 units (U) of activity with >80% inhibition. Seven of 11 lines had 0.018 to 0.095 U of activity; 4 of these were from ID patients. Twelve of 29 lines had TdT activity with 8 having >50% inhibition; TdT values ranged from 0.006 to 0.037 units. The 4 lines without inhibition of TdT ranged from 0.007 to 0.01 U. All of the T cell leukemic lines (including 8392) contained TdT and were inhibited by ATP; the Burkitt's line was negative. Of the ID patients, 3/10 common variable ID, 3/7 ADA + SCID (1 ATP neg.), 1/1 Bruton's and 1/1 Wiskott-Aldrich were positive. All ataxia telangiectasia (4), X-linked agammaglobulemia with hyper-IGM (3), ADA negative SCID (2), and normal cell lines were negative. The presence of TdT in B cell lines from selected ID patients suggests persistence of abnormal lymphocyte differentiation in certain ID states.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Jones, J., Ochs, H. & Meyskens, F. 929 INCOMPLETE LYMPHOCYTE DIFFERENTIATION IN B CELL LINES FROM IMMUNODEFICIENT PATIENTS. Pediatr Res 15 (Suppl 4), 597 (1981). https://doi.org/10.1203/00006450-198104001-00954
Issue Date:
DOI: https://doi.org/10.1203/00006450-198104001-00954