Of note, most of these cases carried nonsense variants in SPTB or ANK1 genes, confirming a previous report in which splenectomy allowed the detection of spectrin and ankyrin defects as the underlying cytoskeletal abnormality in unclassified HS patients (Miraglia del giudice et al., 1997; Mariani et al., 2008; Vercellati et al., 2022). The gene discussed is SPTB; the disease is histiocytic sarcoma.