Most advanced is the Sphk2 inhibitor ABC294640 (Opaganib, Yeliva®), which has received an orphan drug status for the treatment of cholangiocarcinoma and is presently being tested in a phase 2/3 trial for SARS-CoV-2-induced pneumonia (NCT04467840). Here, SPHK2 is linked to cholangiocarcinoma.