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 radiographic examination was done on admission in all the children studied. Indications for the high-resolution computed tomography (HRCT) were considered individually and this examination was performed in four patients. On admission, basic hematological and biochemical tests, evaluation of inflammatory markers and microbiological examinations including blood and bronchial secretion cultures as well as identification of antigens of infectious agents by PCR methods, were performed. Lymphopenia, with low CD3 and CD4 lymphocyte counts (see Table 1 ), low serum levels of major classes of immunoglobulins and flow cytometric evaluation of lymphocyte subsets of peripheral blood and bone marrow have led to the establishing of the diagnosis of a T-B+NK+ SCID in three of the children and a T-B+NK-SCID in the remaining two children. In four of the five affected children, a qualitative PCR examination of CMV-DNA in the peripheral blood was positive, whereas in one of them Pneumocystis jiroveci was identified in bronchial secretions as a co-pathogen. In the next T-B+NK+ SCID 15-month-old female patient, in whom cytomegalovirus infection had not been proven, the human coronavirus HKU1 (hCoV-HKU1) was the only one pathogen identified. In all the children studied, infections with respiratory syncytial virus (RSV) A and B, rhinovirus A and B, adenovirus, metapneumovirus, influenza A (including A H1N1) and B viruses, parainfluenza 1, 2 and 3 viruses, coronavirus oc43 and 229e, Mycoplasma pneumoniae, Chlamydophila pneumoniae, Legionella pneumophila and Pneumocystis jiroveci were excluded on negative PCR examinations of tracheal aspirate samples as well as infections with Epstein-Barr virus (EBV), herpes simplex viruses type 1 and 2 (HSV 1 and 2), hepatitis B and C viruses (HBV, HCV) were excluded on negative PCR examinations of peripheral blood. Infection with human immunodeficiency virus (HIV) was excluded on a negative ELISA test of peripheral blood; however, in an immunocompromised patient a test based on antibody detection is characterized by a low sensitivity. Blood and bronchial secretion cultures did not show any pathological flora, either bacterial or fungal.