Hemophagocytic Lymphohistiocytosis (HLH) is a distinctive disease with high morbidity in which uncontrolled pathologic immune activation occurs either as a familial disorder or as a sporadic condition due to a variety of triggers. This immune disorder results in severe pancytopenia along with extreme inflammation as its clinical presentation. Early detection and treatment may decrease the morbidity and mortality in HLH patients. We report a case of successful haploidentical peripheral blood stem cell (TCR alpha/beta and CD19 B cells depleted) transplantation in familial type 3 hemophagocytic lymphohistiocytosis in a 2 months old baby.
