Clinicopathological aspects of Chediak-Higashi syndrome in the accelerated phase |
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Authors: | Shome Durjoy K Al-Mukharraq Hussain Mahdi Najat Ameen Ghada Farid Eman |
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Institution: | Department of Pathology, Salmaniya Medical Complex, Manama, Bahrain. shome@batelco.com.bh |
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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. |
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