The Clinical Course of COVID-19 Pneumonia in a 19-Year-Old Man on Intravenous Immunoglobulin Replacement Therapy for X-Linked Agammaglobulinemia

Abstract

Patient: Male, 19-year-old Final Diagnosis: COVID-19 Symptoms: Cough • diarrhea • fever • shortness of breath Medication: — Clinical Procedure: — Specialty: Immunology • Infectious Diseases • Pulmonology Objective: Rare co-existance of disease or pathology Background: Since the emergence of coronavirus disease 2019 (COVID-19), patients with the illness have presented with considerable variation in severity. Some infected individuals present mild or no symptoms, while others present severe illness with some fatal outcomes. Multiple lines of management have been suggested for critically ill patients, such as intravenous immunoglobulin (IVIG) and steroids. IVIG is the main treatment for patients with X-linked agammaglobulinemia. Multiple studies have reported that these patients have excellent outcomes when they contract COVID-19. This report describes the clinical course of COVID-19 pneumonia due to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in a 19-year-old man on IVIG replacement therapy for X-linked agammaglobulinemia (XLA). Case Report: A patient with XLA receiving a monthly dose of IVIG and having bronchiectasis managed by prophylactic azithromycin presented with fever, shortness of breath, productive cough, and diarrhea. He was admitted to our hospital with SARS-CoV-2 infection. His treatment course for COVID-19 was uncomplicated and had excellent results. He completed a 10-day course of piperacillin/tazobactam and his symptoms resolved 3 days after admission, without complications, oxygen supplementation, or intensive care unit admission. Conclusions: Patients with XLA have weakened immunity and therefore may present with an infection as a first symptom. This report describes the mild course of COVID-19 pneumonia in an immunologically vulnerable patient with XLA who presented with SARS-CoV-2 infection while undergoing IVIG replacement therapy. Currently, IVIG is one of many supportive immune therapies undergoing clinical evaluation in patients with severe COVID-19.

SARS-CoV-2 T Cell Response in Severe and Fatal COVID-19 in Primary Antibody Deficiency Patients Without Specific Humoral Immunity

“…It is unknown, whether a lack of specific humoral immunity to SARS-CoV-2 per se leads to an increased risk of RNAemia. Based on numerous mild COVID-19 cases in XLA patients (2,(15)(16)(17)(18)(19)(20), at least generally persisting and high viral loads seem unlikely.…”

“…With regard to humoral immunity, early clinical data from XLA patients suggested that lack of SARS-CoV-2 humoral immunity may be sufficiently compensated by innate and T cell immunity in order to prevent severe COVID-19 (2,(15)(16)(17)(18)(19)(20). On the other hand, patients with Good's syndrome, a thymoma-associated hypogammaglobulinemia with B and/or T cell deficiency and lack of specific humoral immune response, were reported to be at significantly increased risk for severe COVID-19 and a high case fatality rate (12).…”

Coronavirus disease 2019 in patients with inborn errors of immunity: lessons learned

“…Reflecting the relative frequency of humoral immunodeficiencies among IEIs, the majority of patients who experienced COVID-19 are affected by antibody deficiency ( n = 330, 51%) [ 5 ▪ , 9 ▪ , 10 , 12 – 17 , 19 , 24 , 26 , 28 , 30 , 33 , 34 , 36 , 37 , 42 , 44 , 45 , 48 , 52 , 53 , 55 , 57 – 62 , 64 , 66 – 69 ]. Among them, 200 have common variable immunodeficiency (CVID, 60% of all antibody deficiencies), 59 have X-linked agammaglobulinemia (XLA, 18%) and eight have autosomal recessive agammaglobulinemia (2%).…”

Mechanisms underlying host defense and disease pathology in response to severe acute respiratory syndrome (SARS)-CoV2 infection: insights from inborn errors of immunity

“…BTK signaling -roles for Ab, myeloid cells, and cytokine production Many cases of SARS-CoV2 infection in XLA patients have been reported [32,35,42 ]. When disease was moderate, patients usually recovered without developing pneumonia [35,55].…”