Respiratory symptoms of COVID-19 in an adolescent patient with WHIM syndrome: a clinical case

In case of coronavirus disease 2019 (COVID-19) in children suffering from primary immunodeficiency, the last one can be an aggravating or a mitigating factor of the course of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. WHIM (warts, hypogammaglobulinemia, infections, and myelokathexis) syndrome is usually classified as severe congenital neutropenia, but most patients have multiple leukocyte deficits, even panleukopenia, and therefore it can also be classified as severe combined immunodeficiency. B-lymphopenia is especially severe, and this probably partly explains the hypogammaglobulinemia. This rare disease, caused by autosomal dominant mutations, is a combined variant of immunodeficiency, which includes myelokathexis, susceptibility to infections, and hypogammaglobulinemia. Myelokathexis is a unique form of acyclic severe congenital neutropenia caused by the accumulation of mature and degenerative neutrophils in the bone marrow. Monocytopenia and lymphopenia, especially B-lymphopenia, also occur. In some patients, there are defects in the development of the cardiovascular, genitourinary and nervous systems, which in general can contribute to the extremely severe course of infectious inflammatory process, in particular due to the SARS-CoV-2. Objective: to analyze the clinical and laboratory peculiarities of coronavirus disease caused by SARS-CoV-2 in immunosuppressed patients on the example of a clinical case of COVID-19 in a child with previously diagnosed WHIM syndrome. Materials and methods. The article presents our own observation of coronavirus disease in a female adolescent suffering from previously verified primary immunodeficiency (WHIM syndrome) in the period after surgical routine correction of patent ductus arteriosus. On the 2nd day of sudden disease onset, the child was hospitalized in moderate condition with signs of airway inflammation as rhinopharyngitis and obstructive bronchitis. Results. Laboratory tests showed leukopenia, absolute neutropenia, increased levels of procalcitonin, C-reactive protein, D-dimer in serum and a reduction of activated partial thromboplastin time. The treatment included hydrobalance protection per os and by infusion, systemic and topical inhalation therapy with a short steroids course, antibacterial therapy as fourth generation cephalosporins, intravenous granulocyte colony stimulating factor, and symptomatic treatment. The girl’s condition became progressively better, she was discharg