Combined umbilical cord blood and bone marrow transplantation from a sibling in a patient with Fanconi anemia

Pawelec, Katarzyna; Boruczkowski, Dariusz; Ołdak, Tomasz; Ussowicz, Marek; Demkow, Urszula; Kałwak, Krzysztof

doi:10.5114/ceji.2013.37749

Cited by 1 publication

(1 citation statement)

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“…Cell-mediated immunity is the main defense against CMV disease, including CD4 T helper cells and interferon gamma (IFN-γ) and interleukin 2 (IL-2) production, CD8 T cell-mediated cytolytic activity, as well as NK cell cytotoxicity. As inadequate immune response of CD4 T cells is a major factor responsible for lack of immune control of CMV infection, therefore, cytomegalovirus is considered to be a major pathogen in severely immunocompromised individuals as infants with SCID [ 8 ] and children with secondary immunodeficiencies, such as recipients of hematopoietic cell transplantation [ 9 , 10 ]. Significant morbidity and mortality in immunodeficient hosts may result from a primary, congenital or acquired infection, occur as a consequence of reactivation of the latent virus, or infection with a new CMV strain.…”

Section: Discussionmentioning

confidence: 99%

Clinical immunology Cytomegalovirus pneumonia as the first manifestation of severe combined immunodeficiency

Szczawińska-Popłonyk¹,

Jończyk‐Potoczna²,

Ossowska³

et al. 2014

cejoi

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Severe combined immunodeficiency (SCID) is characterized by the absence of functional T lymphocytes and impairment of adaptive immunity. While heterogeneity of the genetic background in SCID leads to the variability of immune phenotypes, most of affected newborns appear healthy but within the first few months they develop life-threatening opportunistic respiratory or gastrointestinal tract infections. The objective of the study was to define the presenting features and etiology of infections in children with SCID. We retrospectively reviewed five children in whom the diagnosis of SCID had been established in our pediatric immunology clinic over the last 10-year period. A viral respiratory tract infection was the first manifestation of SCID in all the children studied. Cytomegalovirus (CMV) pneumonia was recognized in as many as 4 cases and coronavirus pulmonary infection was diagnosed in one case, whereas Pneumocystis jiroveci was identified as a co-pathogen in one CMV-infected patient. Severe combined immunodeficiency is a pediatric emergency condition and given the significant impact of pulmonary CMV infection in SCID children, establishing an accurate etiological diagnosis is of essential importance in instituting the specific treatment and improving the outcome.

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Section: Discussionmentioning

confidence: 99%