CHEDIAK-HIGASHI SYNDROME IN ACCELERATED PHASE AT INITIAL PRESENTATION
Jayashree Nadkarni, Thanga Sarvanan, Rashmi Dwivedi
Department of Pediatrics, Gandhi Medical College and Kamla Nehru Hospital, Bhopal, India
Abstract

Chediak - Higashi Syndrome (CHS) is a rare autosomal recessive disease which results from defective degranulation of primary lysosomal granules with delayed microbial killing. Most of the patients with CHS enter into an "accelerated phase" as in our case who manifested by fever, hepatosplenomegaly and lymphadenopathy leading to pancytopenia and bleeding disorders.
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