Figure 4.
Diagnostic algorithm for autoimmune lymphoproliferative syndrome (ALPS). A presumptive diagnosis of ALPS may be rendered based on clinical history and the results of laboratory testing. A definitive diagnosis requires genetic testing and potentially functional assays. Genetic testing can be performed in a stepwise fashion and may incorporate multigene panels. *** = diagnosis depends on results of multigene testing; IL = interleukin; NGS = next generation sequencing; DNT = double negative T; sFAS = somatic FAS; FASL = FAS ligand; CASP10 = Caspase 10.