Browsing by Author "Paiva, A."
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- Phenotypic profile of expanded NK cells in chronic lymphoproliferative disorders: a surrogate marker for NK-cell clonalityPublication . Bárcena, P.; Jara-Acevedo, M.; Tabernero, M.; López, A.; Sánchez, M.; García-Montero, A.; Muñoz-García, N.; Vidriales, M.; Paiva, A.; Lecrevisse, Q.; Lima, M.; Langerak, A.; Böttcher, S.; van Dongen, J.; Orfao, A.; Almeida, J.Currently, the lack of a universal and specific marker of clonality hampers the diagnosis and classification of chronic expansions of natural killer (NK) cells. Here we investigated the utility of flow cytometric detection of aberrant/altered NK-cell phenotypes as a surrogate marker for clonality, in the diagnostic work-up of chronic lymphoproliferative disorders of NK cells (CLPD-NK). For this purpose, a large panel of markers was evaluated by multiparametric flow cytometry on peripheral blood (PB) CD56low NK cells from 60 patients, including 23 subjects with predefined clonal (n = 9) and polyclonal (n = 14) CD56low NK-cell expansions, and 37 with CLPD-NK of undetermined clonality; also, PB samples from 10 healthy adults were included. Clonality was established using the human androgen receptor (HUMARA) assay. Clonal NK cells were found to show decreased expression of CD7, CD11b and CD38, and higher CD2, CD94 and HLADR levels vs. normal NK cells, together with a restricted repertoire of expression of the CD158a, CD158b and CD161 killer-associated receptors. In turn, NK cells from both clonal and polyclonal CLPD-NK showed similar/overlapping phenotypic profiles, except for high and more homogeneous expression of CD94 and HLADR, which was restricted to clonal CLPD-NK. We conclude that the CD94hi/HLADR+ phenotypic profile proved to be a useful surrogate marker for NK-cell clonality.
- Pneumonite intersticial crónicaPublication . Almeida, R.; Reis, G.; Ferreira, C.; Oliveira, M.; Oliveira, D.; Fernandes, P.; Ferreira, P.; Frutuoso, S.; Carreira, L.; Alves, V.; Paiva, A.; Guedes, M.A patologia pulmonar intersticial compreende um grupo de doenças crónicas caracterizadas por alterações das paredes alveolares e perda das unidades funcionais alveolocapilares. São doenças raras nas crianças, na sua maioria de causa desconhecida e revestindo-se habitualmente de uma elevada morbimortalidade, dada a pouca eficácia da terapêutica actualmente disponível. Os autores descrevem o caso clínico de uma criança de 3 anos, previamente saudável, que no contexto de uma infecção respiratória desenvolve um quadro de sibilância e insuficiência respiratória grave, na investigação do qual é diagnosticada uma pneumonite intersticial crónica. Foram tentadas diversas terapêuticas (corticoterapia sistémica, hidroxicloroquina, N-acetilcisteína) sem melhoria evidente.Interstitial lung disease includes a group of chronic diseases characterized by alterations in alveolar walls and loss of functional alveolar-capillary units. These are rare diseases in children, mostly with an unknown cause and associated with a high morbidity and mortality due to insufficient therapeutic effectiveness. The authors report a case of a previously healthy 3 years old child who presented with wheezing and severe respiratory insufficiency following a respiratory infection. The investigation performed led to the diagnosis of chronic interstitial pneumonitis. Several treatments have been tried (corticosteroids,hydroxychloroquine, N-acetylcysteine) without any obvious improvement.