Figure 3.

Diagnostic work-up based on degranulation assays in suspected hemophagocytic lymphohistiocytosis. 2B4, surface molecule on NK cells; AICD, activation-induced cell death; CHS, Chédiak Higashi syndrome; CTL, CD8+ cytotoxic T lymphocyte; FHL, familial hemophagocytic lymphohistiocytosis; GS2, Griscelli syndrome type II; HLH, hemophagocytic lymphohistiocytosis; NK, natural killer; SAP, signaling lymphocytic activation molecule-associated protein; XIAP, X-linked inhibitor of apoptosis. This research was originally published in Blood. Bryceson YT, Pende D, Maul-Pavicic A, Gilmour KC, Ufheil H, Vraetz T, Chiang SC, Marcenaro S, Meazza R, Bondzio I, Walshe D, Janka G, Lehmberg K, Beutel K, zur Stadt U, Binder N, Arico M, Moretta L, Henter JI, Ehl S: A prospective evaluation of degranulation assays in the rapid diagnosis of familial hemophagocytic syndromes. Blood 2012, 119:2754-63. © The American Society of Hematology [51].