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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).
Our patient manifested a classical presentation of FHLH. Such manifestations of HLH can be confused with sepsis, pyrexia of unknown origin, hepatitis or malignancy (14).
Onset of FHLH occurs generally in infancy, but rarely, patients can be symptomatic at later ages, even in adulthood (7).
Patients with parental consanguinity and history of sibling death were accepted as having FHLH. Parental consanguinity was present in all of the patients and history of sibling death in 18 of them.
Familial hemophagocytic lymphohistiocytosis (FHLH) is a rare autosomal recessive disease characterized by fever, hepatosplenomegaly, cytopenia, and hemophagocytosis in the bone marrow or other tissues (1).
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