Clinicopathological aspects of Chediak-Higashi syndrome in the accelerated phase

Authors

• Durjoy K. Shome, Department of Pathology, Salmaniya Medical Complex, PO Box 12, Manama, Bahrain. Tel. +973 279007. Fax. +973 279649. E-mail: shome{at}batelco.com.bh
• Hussain Al-Mukharraq, Department of Pediatrics, Salmaniya Medical Complex, PO Box 12, Manama, Bahrain.
• Najat Mahdi, Department of Pediatrics, Salmaniya Medical Complex, PO Box 12, Manama, Bahrain.
• Ghada Ameen, Department of Pathology, Bahrain Defence Forces Medical Division, Military Hospital, Bahrain.
• Eman Farid, Department of Pathology, Salmaniya Medical Complex, PO Box 12, Manama, Bahrain. Tel. +973 279007. Fax. +973 279649.

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.

Article Type

Case Report

First Page

464

Last Page

466

Recommended Citation

Shome, Durjoy K.; Al-Mukharraq, Hussain; Mahdi, Najat; Ameen, Ghada; and Farid, Eman (2002) "Clinicopathological aspects of Chediak-Higashi syndrome in the accelerated phase," Saudi Medical Journal: Vol. 23: Iss. 4, Article 20.
DOI: https://doi.org/10.15537/1658-3175.1683