Abstract
The diagnosis of severe combined immunodeficiency (SCID) was established in a 5 months old boy based on typical clinical and laboratory findings. Analysis of surface markers, however, revealed a remarkable pattern: CD3 86%, CD4 5%, CD8 62%, TCR-1 38%, TCR-2 47%, CD8+/TCR-1+ 21%, CD8+/TCR-2+ 24%. Thus, the CD8+/TCR-1+ population almost absent in normal blood was extremely elevated to 21% of PBMC. To further characterize these cells and the complementary fractions they were double labelled using commercial monoclonal antibodies to CD8 and TCR-1 and sorted by fluorescence activated cell sorting (FACSTAR PLUS). DNA from sorted cells was next analyzed by Southern Slot using a common delta-specific probe. A monoclonal rearrangemnt (Vδ1 to Jδ1) was exclusively found in the abnormally elevated cells but not in any other population analyzed. Using PCR we next generated a clonospecific probe directed against the NDN sequences of the rearranged fragment. Dot blot hybridization with this probe confirmed Southern blot results. The mother's PBMC contained 5% T cells with CD8+/TCR-1+ phenotype which had an identical rearrangement on Southern blot and reacted with the child's clonospecific probe, in addition, the NDN nucleotide sequences were identical. Because of signs of GvHR skin and liver biopsies were taken. Southern blots of DNA extracted from the skin revealed the presence of the rearranged fragment while only a minimal reaction could be observed with liver DNA. This case shows that monoclonal maternal cells can metastasize diaplacentally into the child if they are not destroyed by the child's immune system and that CD8+/TCR-1+ cells may have a rule in the skin but not in the liver.
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Wahn, V., Heyer, K., Yokata, S. et al. 131 EXPANSION OF A MATERNALLY ACQUIRED MONOCLONAL T CELL POPULATION WITH CD3+/CD8+/TCR-1-PHENOTYPE IN A CHILD WITH SCID AND CHRONIC GvHR POSITIVE TROPISM OF THESE CELLS INTO THE SKIN BUT NOT INTO THE LIVER. Pediatr Res 28, 299 (1990). https://doi.org/10.1203/00006450-199009000-00155
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DOI: https://doi.org/10.1203/00006450-199009000-00155