Clinicopathological aspects of Chediak-Higashi syndrome in the accelerated phase.
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.
Shome
Durjoy K
DK
Department of Pathology, Salmaniya Medical Complex, Manama, Bahrain. shome@batelco.com.bh
Al-Mukharraq
Hussain
H
Mahdi
Najat
N
Ameen
Ghada
G
Farid
Eman
E
D002609:Chediak-Higashi Syndrome / Q000473:pathology* / Q000628:therapy; D005260:Female; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use; D006801:Humans; D007223:Infant
D016179:Granulocyte Colony-Stimulating Factor