Rare case of hemophagocytic disorder: A family with Chediak Higashi Syndrome

Chediak higasi syndrome (chs) is an autosomal recessive disorder characterized by partial occulocutaneous albinism,increased susceptibility to infection,photophobia,a mild bleeding diathesis and a tendency to develop a life-threatening lymphoma like syndrome. many similar cases of this disease with some additional features have been described in the national and international journals. pancytopenia,hepatosplenomegaly,lymphohistiocytic infiltration in bone marrow and the abnormal characteristic granules in leukocytes lead to the diagnosis in the reported case.