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¹A.İ.B.Ü. İzzet Baysal Tıp Fak. İç Hast. A.D. Hematoloji B.D., Gölköy / Bolu
²A.İ.B.Ü. İzzet Baysal Tıp Fak. Göz A.D., Bolu
³Fırat Üni. Tıp Fak. İç Hast. ABD Onkoloji B.D., Elazığ
⁴Fırat Üni. Tıp Fak. Göz Hast. A.D., Elazığ The Hermansky-Pudlak Syndrome (HPS) is a clinical syndrome described as autosomal recessively inherited tyrosinase-positive oculocutaneous albinism with bleeding diathesis and accumulation of ceroid in tissues. A patient with bleeding diathesis and given thrombocyte suspension presented with amaurosis fugax, consulted and diagnosed as HPS. We reported the characteristic findings of the HPS that can be easily overlooked in patients with albinism. Keywords : Hermansky-Pudlak Syndrome, Ocular albinism, Platelet disfunction