RT Journal Article
SR Electronic
T1 Clinicopathological aspects of Chediak-Higashi syndrome in the accelerated phase
JF Saudi Medical Journal
JO Saudi Med J
FD Prince Sultan Military Medical City
SP 464
OP 466
VO 23
IS 4
A1 Durjoy K. Shome
A1 Hussain Al-Mukharraq
A1 Najat Mahdi
A1 Ghada Ameen
A1 Eman Farid
YR 2002
UL http://smj.org.sa/content/23/4/464.abstract
AB 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.