Multiple mutations in GP Ibα, GP Ibβ, or GP IX leading to clinical expression of BSS are found including missense, nonsense mutations and frame shift insertions or deletions resulting in defect in expression of the GP Ib-IX-V complex on platelets.4,5 BSS cases with isolated GPV gene mutation have not been reported in literature.6 This evidence concerns the gene GP1BA and Bernard-Soulier syndrome.