Cytomegalovirus pneumonia as the first manifestation of severe combined immunodeficiency

Szczawinska-Poplonyk, Aleksandra; Jonczyk-Potoczna, Katarzyna; Ossowska, Lidia; Breborowicz, Anna; Bartkowska-Sniatkowska, Alicja; Wachowiak, Jacek

A chest X-ray of a 10-month old boy with a T-B+NK-SCID and CMV infection, showing extensive inflammatory infiltrations, most intensive in the perihilar areas of both lungs, with blurred borders of the heart shape and focal and peripheral emphysema ma, such as abscesses or pneumatoceles were not observed in any of the children studied. The radiographic presentation of CMV-related pneumopathy in a child with SCID is shown on Fig. 1 . Unfortunately, none of these children had a HLAmatched sibling donor and haploidentical hematopoietic cell transplantation (HCT) was performed in three of the children. In one of these children, post-transplantation chronic CMV infection and graft versus host disease contributed to a fatal outcome. In the next two children with T-B+NK+ SCID, one with CMV coexisting with Pneumocystis jiroveci infection and in another one with hCoV-HKU1 infection, who developed the most severe symptoms of respiratory and circulatory insufficiency and were hospitalized in the intensive care unit, HCT was rendered impossible because of their critical state. These children died because of the multiorgan failure despite intensive multidirectional antiviral (including ganciclovir or acyclovir), antibacterial and antifungal pharmacotherapy, immunoglobulin infusions and prophylaxis against Pneumocystis jiroveci infection.