ABSTRACT
Identi cation of Subset Abnormalities in Primary Immunode ciency Disease ................................................. 272 9.3.1.2 Th−Tc−NK−B+ X-Linked Severe Combined
Immunode ciency ............................................................... 276 9.3.1.3 Th−Tc−NK−B+, Autosomal Severe Combined Immunode ciency (JAK3) .................................................. 277 9.3.1.4 Th−Tc−B+NK− (CD45) .................................................... 277 9.3.1.5 Th−Tc−B+NK+ (IL-7R Alpha, CD3 Delta,
and CD3 Zeta) ..................................................................... 277 9.3.1.6 Th−Tc−B−NK+ (Rag1, Rag2, Lig IV, Artemis) ............... 277 9.3.1.7 Th−Tc−B−NK− (Adenosine Deaminase Gene) ................ 279 9.3.1.8 Th+Tc+B+NK− (IL-2R/IL-15Rβ) ..................................... 279 9.3.1.9 Omenn Syndrome ............................................................... 279 9.3.1.10 T-Cell Receptor V-Beta Repertoire Analysis in CD4 and CD8 T-Cell Subsets ...................................................... 279 9.3.1.11 Summary of Severe Combined Immunode ciency ............282 9.3.1.12 Hyper IgM Syndromes: CD40 Ligand, CD40
Receptor-Group I ............................................................... 283 9.3.1.13 Measurement of CD40 Ligand on in Vitro-Activated T-Helper Cells and CD40 Receptor on B Cells .................. 283
9.3.1.14 Abnormal HLA-DR Expression (Omenn SyndromeAbnormally Elevated and MHC Class II De ciencyAbnormally Decreased) ......................................................284
9.3.1.15 Decreased CD8+ T Cells (TAP-1/2 De ciency, ZAP-70 De ciency) .............................................................287 9.3.1.16 Decreased CD4+ T Cells ...................................................287
