Chédiak-Higashi Syndrome: Case Report of Three Patients in an Iraqi Family in Tikrit City

Abstract

Chediak-Higashi syndrome is a rare, inherited, multisystem disorder affecting the immune system, pigmentation, and neurologic functions, and characterized by recurrent infections, giant cytoplasmic granules, and oculocutaneous albinism. Here a case report of three patients in an Iraqi family treated in pediatric ward in Tikrit teaching hospital, Tikrit city. Three patients had oculocutaneous albinism, recurrent infections and giant cytoplasmic granules in the leukocytes. They are members of a poor consanguineous parents. They have different presentation regarding the onset, the clinical signs, and the subsequent coarse of the illness. Tow patients died of infectious complications during the accelerated phase and the other girl is still in the resting phase. Therapy included ascorbic acid and antibiotics. Chemotherapy was used for the accelerated phase in two patients. Early diagnosis and therapy of CHS is needed. Bone marrow transplantation should be indicated before the accelerated phase of the disease has developed.