Two X-linked agammaglobulinemia patients develop pneumonia as COVID-19 manifestation but recover.

The recent SARS-Cov2 pandemic, which has recently affected Italy since February 21, constitutes a threat for normal subjects, as the Coronavirus Disease 19 (COVID19) can manifest with a broad spectrum of clinical phenotypes ranging from asymptomatic cases to pneumonia or even death. There is evidence that older age and several comorbidities can affect the risk to develop severe pneumonia and possibly the need of mechanic ventilation in subjects infected with SARS-Cov2. Therefore, we evaluated the outcome of SARS-Cov2 infection in patients with inborn errors of immunity (IEI) such as X linked agammaglobulinemia (XLA).When the SARS-Cov2 epidemic has reached Italy, we have activated a surveillance protocol of patients with IEI, to perform SARS-Cov2 search by nasopharyngeal swab in patients presenting with symptoms which could be a manifestations of COVID-19, such as fever, cough, diarrhea or vomiting.We describe two patients with X-linked agammaglobulinemia (XLA) of 34 and 26 years of age with complete absence of B cells from peripheral blood who developed COVID-19, as diagnosed by SARS-Cov-2 detection by nasopharyngeal swab, while receiving immunoglobulin infusions. Both patients developed interstitial pneumonia characterized by fever, cough and anorexia and associated with elevation of CRP and ferritin, but have never required oxygen ventilation or intensive care.Our report suggests that XLA patients might present high risk to develop pneumonia after SARS-Cov2 infection, but can recover from infection, suggesting that B cell response might be important, but not strictly required to overcome the disease. However, there is need of larger observational studies to extend these conclusions to other patients with similar genetic immune defects.