Primary haemophagocytic lymphohistiocytosis (Chédiak-Higashi Syndrome) triggered by acute SARS-CoV-2 infection in a six-week-old infant.

Abstract

Haemophagocytic lymphohistiocytosis (HLH) is a rare haematologic disorder caused by dysregulation of the immune system, resulting in an overproduction of cytokines and an unrestrained haemophagocytosis. Primary (inherited) forms of HLH are linked to genetic mutations and have predominantly been described in the paediatric population. Secondary (acquired) forms of HLH may be triggered by infections or may develop in the context of autoimmune disease and malignancy. We present a case of primary HLH triggered by SARS-CoV-2 infection in a 6-week-old infant later diagnosed with Chédiak-Higashi syndrome (CHS).