FHLHFamilial Hemophagocytic Lymphohistiocytosis
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Based on the above investigations a diagnosis of FHLH was made.
FHLH is rare AR disorder and have poor prognosis and tends to occur in early infancy (4).
FHLH results from mutation of a protein that mediates the cytotoxic activity of NK cells and T cells called perforin with a locus on chromosome 10 (9).
Patients with parental consanguinity and history of sibling death were accepted as having FHLH.
Many reports on FHLH in Turkish patients justify this conclusion (11-13).
As is known, the presence of infection does not discriminate FHLH from the acquired form.