CD8 (+)/V beta 5.1(+) large granular lymphocyte leukemia associated with autoimmune cytopenias, rheumatoid arthritis and vascular mammary skin lesions: successful response to 2‐deoxycoformycin.

Hematol Oncol. 2002 Jun;20(2):87-93. Cd8(+)/V beta 5.1(+) large granular lymphocyte leukemia associated with autoimmune cytopenias, rheumatoid arthritis and vascular mammary skin lesions: successful response to 2-deoxycoformycin. Granjo E, Lima M, Correia T, Lisboa C, Magalhães C, Cunha N, Teixeira MA, Queirós ML, Candeias J, Matutes E. Department of Clinical Haematology, Hospital Geral de São João, Porto, Portugal. [email protected] Abstract We report a case of CD8(+)/V beta 5.1(+) T-cell large granular lymphocyte leukemia (T-LGL leukemia) presenting with mild lymphocytosis, severe autoimmune neutropenia, thrombocytopenia, polyarthritis and recurrent infections with a chronic disease course. Immunophenotyping showed an expansion of CD3(+)/TCR alpha beta(+)/CD8(+bright)/CD11c(+)/CD57(-)/CD56(-) large granular lymphocytes with expression of the TCR-V beta 5.1 family. Southern blot analysis revealed a clonal rearrangement of the TCR beta-chain gene. Hematopoietic growth factors, high dose intravenous immunoglobulin and corticosteroids were of limited therapeutic benefit to correct the cytopenias. During the disease course, the patient developed a severe cutaneous leg ulcer and bilateral vascular mammary skin lesions. Treatment with 2-deoxycoformycin resulted in both clinical and hematological complete responses, including the resolution of vascular skin lesions. Combined immuno-staining with relevant T-cell associated and anti-TCR-V beta monoclonal antibodies proved to be a sensitive method to assess the therapeutic effect of 2-deoxycoformicin and to evaluate the residual disease. Copyright 2002 John Wiley & Sons, Ltd. PMID: 12111871 [PubMed - indexed for MEDLIN

Intraclonal diversity in a Sezary syndrome with a differential response to 2‐deoxycoformycin of the two lymphoma cell populations

Br J Haematol. 2002 Dec;119(3):629-33. Intraclonal diversity in a Sezary syndrome with a differential response to 2-deoxycoformycin of the two lymphoma cell populations. Granjo E, Lima M, Lopes JM, Cunha N, Teixeira Mdos A, Santos F, Candeias J, Resende C, Santos AH, Balanzategui A, Orfão A, Matutes E. Department of Clinical Haematology, Hospital Geral de São João, Porto, Portugal. [email protected] Abstract We report a case of Sezary syndrome with two abnormal CD4+ T-cell populations detected in the peripheral blood by flow cytometry immunophenotyping and DNA cell content, suggesting a biclonal T-cell lymphoproliferative disorder. Despite these findings, molecular analysis of the T-cell receptor genes was consistent with a monoclonal T-cell proliferation, supporting the existence of intraclonal diversity rather than a true biclonal disease. The patient achieved a transient response with 2-deoxycoformycin, with a selective decrease of the larger/hyperploid T-cell population; later on, an increased representation of this T-cell population was observed concomitantly with clinical relapse. PMID: 12437636 [PubMed - indexed for MEDLINE

Abnormal NK cell lymphocytosis detected after splenectomy: association with repeated infections, relapsing neutropenia, and persistent polyclonal B-cell proliferation

Abnormal NK cell lymphocytosis detected after splenectomy: association with repeated infections, relapsing neutropenia, and persistent polyclonal B-cell proliferation. Granjo E, Lima M, Fraga M, Santos F, Magalhães C, Queirós ML, Moreira I, Rocha S, Silva AS, Rebelo I, Quintanilha A, Ribeiro ML, Candeias J, Orfão A. Department of Hematology, Hospital S. João, Porto, Portugal. [email protected] Abstract We report the case of a boy with hereditary spherocytosis who presented with mild microcytic hypochromic anemia and recurrent leg ulcers that had been present since childhood. Chronic natural killer (NK) cell and B-cell lymphocytosis was detected 1 year after therapeutic splenectomy during investigation of recurrent episodes of neutropenia and persistent lymphocytosis. NK cells proved to be abnormal at immunophenotyping studies, and B-cells were polyclonal and displayed a normal immunophenotype. Genotypic analysis of T-cell receptor (TCR)-beta and TCR-gamma genes showed a germ-line pattern. The clinical course of this patient was characterized by multiple pulmonary infections and amygdalitis. We discuss the potential roles of persistent immune stimulation due to chronic hemolysis and severe leg ulcers and of splenectomy in the origin of NK cell lymphocytosis and the relationship between NK cells and recurrent infections, relapsing neutropenia, and polyclonal B-cell response

Prospective Study of Loss of Health-Related Quality Adjusted Life Years in Children and their Families due to Uncomplicated and Hospitalised Varicella

Introduction and aims: Although usually benign, varicella can lead to serious complications and sometimes long-term sequelae. Vaccines are safe and effective but not yet included in immunisation programmes in many countries. We aimed to quantify the impact on health-related quality of life (HRQoL) in terms of quality-adjusted life years (QALY) in children with varicella and their families, key to assessing cost-utility in countries with low mortality due to this infection. Methods: Children with varicella in the community and admitted to hospitals in Portugal were included over 18 months from January 2019. Children's and carers' HRQoL losses were assessed prospectively using standard multi-attribute utility instruments for measuring HRQoL (EQ-5D and CHU9D), from presentation to recovery, allowing the calculation of QALYs. Results: Among 109 families with children with varicella recruited from attendees at a pediatric emergency service (community arm), the mean HRQoL loss/child was 2.0 days (95 % CI 1.9-2.2, n = 101) (mean 5.4 QALYs/1000 children (95 % CI 5.3-6.1) and 1.3 days/primary carer (95 % CI 1.2-1.6, n = 103) (mean 3.6 QALYs /1000 carers (95 % CI 3.4-4.4). Among 114 families with children admitted to hospital because of severe varicella or a complication (hospital arm), the mean HRQoL loss/child was 9.8 days (95 % CI 9.4-10.6, n = 114) (mean 26.8 QALYs /1000 children (95 % CI 25.8-29.0) and 8.5 days/primary carer (95 % CI 7.4-9.6, n = 114) (mean 23.4 QALYs/1000 carers (95 % CI 20.3-26.2). Mean QALY losses/1000 patients were particularly high for bone and joint infections [67.5 (95 % CI 43.9-97.6)]. Estimates for children's QALYs lost using the CHU9D tool were well correlated with those obtained using EQ-5D, but substantially lower. Conclusions: The impact of varicella on HRQoL is substantial. We report the first measurements of QALYs lost in hospitalised children and in the families of children both in the community and admitted to hospital, providing important information to guide vaccination policy recommendations.info:eu-repo/semantics/publishedVersio

Perspectivas psicossociais para o estudo do cotidiano de trabalho

Cette étude visait à analyser le débat et l'approfondissement théorique et méthodologique développé dans les études sur la vie quotidienne au travail. L'investigation a démarré à partir du dialogue interdisciplinaire entre l'anthropologie du travail, l'ergologie et la psychologie sociale du travail et il a systématisé les contributions mutuels entre ces domaines scientifiques, leurs approches et leurs départs de la thématisation et l'appropriation de l'activité humaine au travail sous la perspective du quotidien. L'analyse comparative a mis en évidence des axes qui différencient les manières pour approcher l'objet, qui se réfèrent aux relations établies entre savoirs pratiques et technico-scientifiques et aux manières d'intervention sur les situations de travail. Les approches comparatives partagent l'intérêt de connaître le vrai travail, la valeur attribuée à les savoirs des travailleurs et la sensibilité aux dimensions micropolitiques du quotidien. On en conclut que la localisation dans chacun de ces axes dépend d'une plus grande ou plus petite affiliation à les traditions d'anthropologie ou d'ergonomie.Este estudio tuvo como objetivo analizar el debate y la profundización teórica y metodológica desarrollada en los estudios acerca de la vida cotidiana en el trabajo. La investigación partió del diálogo interdisciplinario entre la antropología del trabajo, la ergología y la psicología social del trabajo y sistematizó las contribuciones mutuas entre eses campos científicos, sus aproximaciones y sus alejamientos de la tematización y la apropiación de la actividad humana en el trabajo bajo la perspectiva del cotidiano. El análisis comparativo destacó ejes que diferencian las formas de aproximación al objeto, que se refieren a las relaciones establecidas entre saberes prácticos y técnico-científicos y a los modos de intervención en las situaciones de trabajo. Los abordajes comparativos comparten el interés en conocer el trabajo real, el valor dado a los conocimientos de los trabajadores y la sensibilidad a las dimensiones micropolíticas del cotidiano. Se concluye que la localización en cada uno de eses ejes depende de la mayor o menor filiación a las tradiciones de la antropología o de la ergonomía.Este estudo teve por objetivo analisar o debate e o aprofundamento teórico e metodológico desenvolvido nos estudos sobre a vida cotidiana no trabalho. A investigação partiu do diálogo interdisciplinar entre a antropologia do trabalho, a ergologia e a psicologia social do trabalho e sistematizou as contribuições mútuas entre esses campos científicos, suas aproximações e seus afastamentos da tematização e da apropriação da atividade humana no trabalho sob a perspectiva do cotidiano. A análise comparativa destacou eixos que diferenciam as formas de aproximação ao objeto, que se referem às relações estabelecidas entre saberes práticos e técnico-científicos e aos modos de intervenção sobre as situações de trabalho. As abordagens comparativas compartilham o interesse em conhecer o trabalho real, o valor conferido aos conhecimentos dos trabalhadores e a sensibilidade às dimensões micropolíticas do cotidiano. Conclui-se que a localização em cada um desses eixos depende da maior ou menor filiação às tradições da antropologia ou da ergonomia.This study aimed to analyze the debate and the theoretical and methodological deepening of studies on daily life work. The investigation started from the interdisciplinary dialogue among anthropology of work, ergology, and social psychology of work and it systematized the mutual contributions among these scientific fields, their approximations and their departures from the thematization and appropriation of human activity at work from the perspective of daily life. The comparative analysis highlighted axes which differentiate the ways for approaching the object, that refer to the relations established between practical and technical-scientific kinds of knowledge and the modes of intervening on work situations. Comparative approaches share the interest in knowing the real work, the value attributed to workers' knowledge and sensitivity to the micropolitical dimensions of daily life. One concludes that the location in each of these axes depends on the greater or lesser affiliation to the traditions of anthropology or ergonomics