428: A PERSISTENT CASE OF COVID-19 IN A PATIENT WITH COMPLETE DIGEORGE SYNDROME

Abstract

Introduction: DiGeorge Syndrome is a relatively common microdeletion syndrome defined by a deletion in chromosome 22 at position 22q11.2 and clinically characterized by T-cell deficiency, unique facial features, congenital heart disease and hypocalcemia. Complete DiGeorge Syndrome refers to a subset that is athymic (< 1%) without any T cell function. The adaptive immune response is then disturbed leading to an increased susceptibility to infections given the lack of cytotoxic response to infected cells. In this population, pathogens can persist and have unique presentations due to the lack of an adaptive immune response. Description: We present a case of persistent COVID-19 in a 3-year-old male with complete DiGeorge syndrome. Two months prior to presentation, he was treated with remdesivir for a symptomatic COVID-19 infection. He later represented with severe persistent hypocalcemia despite aggressive IV electrolyte repletion as well as a new acute hemolytic anemia with positive Coombs and concern for Graft vs Host disease. During his hospital course, he had worsening respiratory status leading to re-intubation. Bronchoscopy with BAL showed significant SARS-CoV-2 viral load and he was started on a repeat course of remdesivir and convalescent plasma. Two weeks after initial bronchoscopy, repeat BAL samples showed unchanged SARS-CoV-2 viral load leading to another course of remdesivir and convalescent plasma. After a month of invasive ventilation, he was able to be successfully extubated and eventually discharged from the hospital, despite having persistently an elevated SARS-CoV-2 viral load and polymerase chain reaction testing positivity. Discussion: This case illustrates a unique situation of COVID-19 in a patient without an adaptive immune response due to complete DiGeorge Syndrome. This patient population is athymic without any T cell function leading to a lack of cytotoxic response and a susceptibility to infections due to the inability to clear infected cells. With COVID’s high mutation rate leading to multiple new strains, we present a case illustrating the possibility of new strain mutations developing due to persistent COVID in a patient with an inability to clear the viral infection.