Hemophagocytic Lymphohistiocytosis in Children with Griscelli Syndrome Type 2 : Genetic, Laboratory findings and Treatment

Abstract

Griscelli syndrome is a rare autosomal recessive inherited syndrome that causes immunodeficiency. Hemophagocytic lymphohistiocytosis (HLH), which is characterized by high mortality, may develop due to Griscelli syndrome type 2 (GS2). We aimed to share our experience in diagnosis and treatment methods of patients who developed HLH secondary to GS2. GS2 patients