APOB and familial hypercholesterolemia: As such, evinacumab has been initially labeled for patients with homozygous familial hypercholesterolemia [54], which is a highly rare disorder (approximately 1 in 300,000) that classically results from homozygous or compound heterozygous pathogenic variants in LDLR, APOB, or PCSK9 leading to minimal intact LDL receptor function [52].