Association of Chediak-Higashi Syndrome and Human Immunodeficiency Virus Type 1 Acute Infection

Ariani V Bernarchi, Gabriela M

Abstract

Context: The Chediak- Higashi syndrome (CHS) is a rare autosomal and recessive disease associated with a genetic alteration of the lysosomal trafficking regulator (LYST) gene (CHS1/LYST). The LYST protein interacts with some cytoplasmic proteins that play an important role in vesicular transport regulation signal transduction that leads to a decreased phagocytosis, which results in recurrent pyogenic infections, partial albinism and peripheral neuropathy. The association between CHS and infection by human immunodeficiency virus type 1 (HIV-1) is rare. The aim of this study is to report the case of a patient with CHS and had incidental diagnosis of acute HIV-1 infection during hospitalization for treatment of a urological disorder