ETV6-related thrombocytopenia (ETV6-RT) is an autosomal dominant form of inherited thrombocytopenia (IT) caused by mutations in ETV6 (ETS variant 6) gene [1], encoding a master hematopoietic transcriptional repressor structured in three functional domains: the N-terminal pointed domain (PNT), the regulatory domain (or central regulatory domain CRD), and C-terminal DNA-binding domain (ETS). Here, ETV6 is linked to inherited thrombocytopenia.