title: Clinicopathological aspects of Chediak-Higashi syndrome in the accelerated phase.
abstract: This report describes clinical and laboratory features of a case of Chediak-Higashi syndrome that presented in the accelerated phase of the disorder. This female infant presented with a fever, marked neutropenia, large cytoplasmic granules in leukocytes and a constellation of features that suggested a virus-associated hemophagocytic syndrome. The clinical course was marked by limited response to the therapeutic agents that included ascorbate, cytotoxic agents and granulocyte colony-stimulating factor.
authors.affiliation: Department of Pathology, Salmaniya Medical Complex, Manama, Bahrain. shome@batelco.com.bh
authors.forename: Durjoy K
authors.initials: DK
authors.lastname: Shome
authors.forename: Hussain
authors.initials: H
authors.lastname: Al-Mukharraq
authors.forename: Najat
authors.initials: N
authors.lastname: Mahdi
authors.forename: Ghada
authors.initials: G
authors.lastname: Ameen
authors.forename: Eman
authors.initials: E
authors.lastname: Farid
mesh_terms: D002609:Chediak-Higashi Syndrome / Q000473:pathology* / Q000628:therapy; D005260:Female; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use; D006801:Humans; D007223:Infant
chemical_list: D016179:Granulocyte Colony-Stimulating Factor