Browsing by Author "Coutinho, J."
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- BIO-SIMILARES EM ONCOLOGIAPublication . Barroso, S.; Coutinho, J.; Damasceno, M.; Dinis, J.; Lacerda, J.; Gervásio, H.; Costa, F.; Pereira, A.; Parreira, A.; Príncipe, F.; Rodrigues, H.; Sá, A.; Teixeira, A.
- Endovascular Treatment of Aortic Aneurysms and Blood Transfusion. What do We Need?Publication . Machado, R.; Loureiro, L.; Antunes, I.; Coutinho, J.; Almeida, R.INTRODUCTION: Comparatively to open repair, endovascular aneurysm repair has reduced transfusion rates but thereâs no recommendation about number of red blood cells units to be crossmatched preoperatively. Our aim is contribute to the analysis of red blood cells units needs in endovascular and hybrid aortic aneurysm repair and developing a protocol for maximum surgical blood orders schedule. MATERIAL AND METHODS: We retrospectively analyzed our prospective database of elective endovascular aneurysm repair from 2001 to 2012. We analyzed patients' age, gender, ASA classification, maximum surgical blood orders schedule, red blood cells units transfused and timings, types of endoprosthesis, red blood cells units consumption/endoprosthesis' type ratio, crossmatch to transfusion ratio, conversion to open repair, hemoglobin concentrations before surgery and discharge. RESULTS: We selected 187 patients, 90% men, mean age 73.1, ASA mode III. The endoprosthesis were aorto-bi-iliac in 71%, aorto-uni-iliac in 23% and thoracic in 6%. Of these, 72,6% of the patients did not require blood transfusion. We transfused 171 red blood cells units. Crossmatch to transfusion ratio was 10.1 until 2010 and 7.3 after. The ratio of red blood cells units consumption/endoprosthesis in the first 24 hours was 0.21 red blood cells units/aorto-bi-iliac, 0.46 red blood cells units/aorto-uni-iliac, 0.8 red blood cells units/thoracic, 1.3 red blood cells units/hybrid-thoracic and 2 red blood cells units/hybrid-aorto-bi-iliac. A statistical correlation was observed between red blood cells units transfused postoperatively and type of endoprosthesis (p < 0.001) and between ASA classification and red blood cells units transfused after 24 hours (p < 0.01). DISCUSSION: Guidelines from the British Society of Haematology are based on a crossmatch to transfusion ratio of 2:1. Our crossmatch to transfusion ratio was 10.1 until 2010 and 7.3 from 2011 to 2012. CONCLUSION: These results changed our policy of maximum surgical blood orders schedule for endovascular aneurysm repair. We now type and screen aorto-bi-iliac and aorto-uni-iliac. We crossmatch two red blood cells units for thoracic, three red blood cells units for hybrid thoracic and four red blood cells units for hybrid abdominal procedures. This may lead to financial savings, improved efficiency and reduce workload in hematology department.
- Increased red cell distribution width in Fanconi anemia: a novel marker of stress erythropoiesisPublication . Sousa, R.; Gonçalves, C.; Guerra, I.; Costa, E.; Fernandes, A.; Bom-Sucesso, M.; Azevedo, J.; Rodriguez, A.; Rius, R.; Seabra, C.; Ferreira, F.; Ribeiro, L.; Ferrão, A.; Castedo, S.; Cleto, E.; Coutinho, J.; Carvalho, F.; Barbot, J.; Porto, B.BACKGROUND: Red cell distribution width (RDW), a classical parameter used in the differential diagnosis of anemia, has recently been recognized as a marker of chronic inflammation and high levels of oxidative stress (OS). Fanconi anemia (FA) is a genetic disorder associated to redox imbalance and dysfunctional response to OS. Clinically, it is characterized by progressive bone marrow failure, which remains the primary cause of morbidity and mortality. Macrocytosis and increased fetal hemoglobin, two indicators of bone marrow stress erythropoiesis, are generally the first hematological manifestations to appear in FA. However, the significance of RDW and its possible relation to stress erythropoiesis have never been explored in FA. In the present study we analyzed routine complete blood counts from 34 FA patients and evaluated RDW, correlating with the hematological parameters most consistently associated with the FA phenotype. RESULTS: We showed, for the first time, that RDW is significantly increased in FA. We also showed that increased RDW is correlated with thrombocytopenia, neutropenia and, most importantly, highly correlated with anemia. Analyzing sequential hemograms from 3 FA patients with different clinical outcomes, during 10 years follow-up, we confirmed a consistent association between increased RDW and decreased hemoglobin, which supports the postulated importance of RDW in the evaluation of hematological disease progression. CONCLUSIONS: This study shows, for the first time, that RDW is significantly increased in FA, and this increment is correlated with neutropenia, thrombocytopenia, and highly correlated with anemia. According to the present results, it is suggested that increased RDW can be a novel marker of stress erythropoiesis in FA.
- KIT D816V Positive Acute Mast Cell Leukemia Associated with Normal Karyotype Acute Myeloid LeukemiaPublication . Lopes, M.; Teixeira, M.; Casais, C.; Mesquita, V.; Seabra, P.; Cabral, R.; Palla-Garcia, J.; Lau, C.; Rodrigues, J.; Jara-Acevedo, M.; Freitas, I.; Vizcaíno, J.; Coutinho, J.; Escribano, L.; Orfao, A.; Lima, M.Introduction: Mast cell (MC) leukemia (MCL) is extremely rare. We present a case of MCL diagnosed concomitantly with acute myeloblastic leukemia (AML). Case Report: A 41-year-old woman presented with asthenia, anorexia, fever, epigastralgia, and diarrhea. She had a maculopapular skin rash, hepatosplenomegaly, retroperitoneal adenopathies, pancytopenia, 6% blast cells (BC) and 20% MC in the peripheral blood, elevated lactate dehydrogenase, cholestasis, hypoalbuminemia, hypogammaglobulinemia, and increased serum tryptase (184 μg/L). The bone marrow (BM) smears showed 24% myeloblasts, 17% promyelocytes, and 16% abnormal toluidine blue positive MC, and flow cytometry revealed 12% myeloid BC, 34% aberrant promyelocytes, a maturation blockage at the myeloblast/promyelocyte level, and 16% abnormal CD2-CD25+ MC. The BM karyotype was normal, and the KIT D816V mutation was positive in BM cells. The diagnosis of MCL associated with AML was assumed. The patient received corticosteroids, disodium cromoglycate, cladribine, idarubicin and cytosine arabinoside, high-dose cytosine arabinoside, and hematopoietic stem cell transplantation (HSCT). The outcome was favorable, with complete hematological remission two years after diagnosis and one year after HSCT. Conclusions: This case emphasizes the need of an exhaustive laboratory evaluation for the concomitant diagnosis of MCL and AML, and the therapeutic options.
- OS NÍVEIS DE EXPRESSÃO DE CD52 NAS CÉLULAS DE SÉZARY PODERÃO SER ÚTEIS PARA AJUDAR A PREVER A RESPOSTA DOS DOENTES COM SÍNDROME DE SÉZARY AO TRATAMENTO COM ALEMTUZUMAB?Publication . Fernandes, I.; Gonçalves, M.; Lau, C.; Teixeira, M. A.; Gonçalves, C.; Xavier, L.; Coutinho, J.; Selores, M.; Alves, R.; Lima, M.OS NÍVEIS DE EXPRESSÃO DE CD52 NAS CÉLULAS DE SÉZARY PODERÃO SER ÚTEIS PARA AJUDAR A PREVER A RESPOSTA DOS DOENTES COM SÍNDROME DE SÉZARY AO TRATAMENTO COM ALEMTUZUMAB? Iolanda Fernandes1, Marta Gonçalves2, Catarina Lau2, Maria dos Anjos Teixeira2, Cristina Gonçalves2, Luciana Xavier2, Jorge Coutinho2, Manuela Selores1, Rosário Alves1,3,4, Margarida Lima2,3,4 1Serviço de Dermatologia, 2Serviço de Hematologia Clínica, 3Consulta Multidisciplinar de Linfomas Cutâneos, HSA/CHP, 4UMIB/ICBAS/UP. Hospital de Santo António, Centro Hospitalar do Porto (HSA/CHP), Porto. Unidade Multidisciplinar de Investigação Biomédica, Instituto de Ciências Biomédicas Abel Salazar, Universidade do Porto (UMIB/ICBAS/UP), Porto. Introdução O Alemtuzumab (Altz) é um anticorpo monoclonal (AcMo) humanizado específico para o CD52, expresso em diferentes níveis na maioria dos leucócitos, incluindo linfócitos T e B, células NK, monócitos e neutrófilos. Estudos recentes mostraram que o Altz é eficaz no tratamento de neoplasias de células T periféricas, incluindo a Síndrome de Sezary (SS), uma forma rara de linfoma T cutâneo. Embora alguns doentes com SS sejam refractários ao tratamento com Altz, até ao momento não há estudos que avaliem a relação entre a expressão de CD52 nas Células de Sezary (CS) e a resposta à terapêutica. Objectivos Avaliar se a resposta ao tratamento com Altz tem relação com os níveis de expressão de CD52 nas CS. Material e métodos Estudamos a expressão de CD52 nas CS em 5 doentes com SS tratados com Altz subcutâneo em baixa dose, comparando-a com a observada nos restantes leucócitos, em particular nos linfócitos T CD4 normais. As CS foram caracterizadas por citometria de fluxo usando AcMo específicos para diferentes moléculas expressas nos linfócitos T, assim como para diferentes famílias de regiões variáveis da cadeia beta do receptor da célula T, e quantificadas no sangue antes e diferentes tempos após o início do tratamento. Resultados Quatro doentes tiveram boa resposta ao tratamento com Altz, com diminuição acentuada da eritrodermia, do prurido e das CS circulantes. Nesses casos, as CS expressavam CD52 com intensidade forte, igual ou superior à observada nos linfócitos T CD4+ normais residuais. Um dos doentes não respondeu ao tratamento, nem do ponto de vista clínico nem do ponto de vista analítico. Neste caso, as CS expressavam CD52 com intensidade muito fraca, inferior à observada nos neutrófilos, mas os níveis de expressão de CD52 nos restantes leucócitos eram normais. Conclusões Os nossos resultados confirmam os estudos prévios que indicam que o Altz é eficaz no tratamento da maioria dos doentes com SS. Para além disso, indicam que embora na maioria dos casos de SS as CS expressem CD52 com intensidade forte, em alguns casos apresentam uma deficiência selectiva desta molécula. Sugerem ainda que a resposta ao tratamento com Altz está relacionada com os níveis de expressão de CD52 nas CS e que esta deve ser avaliada em todos os doentes propostos para tratamento com este AcMo. É necessário estudar mais casos para determinar se a expressão de CD52 nas CS deve ser usada como biomarcador preditivo da resposta à terapêutica com Altz. Apresentador: Iolanda Fernandes, Médica Interna de Dermatovenerologia, Serviço de Dermatologia, HSA/CHP.
- A pilot study on the usefulness of peripheral blood flow cytometry for the diagnosis of lower risk myelodysplastic syndromes: the "MDS thermometer"Publication . Aires, A.; Teixeira, M.; Lau, C.; Moreira, C.; Spínola, A.; Mota, A.; Freitas, I.; Coutinho, J.; Lima, M.Background: Immunophenotypic analysis of the bone marrow (BM) cells has proven to be helpful in the diagnosis of Myelodysplastic Syndromes (MDS). However, the usefulness of flow cytometry (FCM) for the detection of myelodysplasia in the peripheral blood (PB) still needs to be investigated. The aim of this pilot study was to evaluate the value of FCM-based PB neutrophil and monocyte immunophenotyping for the diagnosis of lower risk MDS (LR-MDS). Methods: We evaluated by 8-color FCM the expression of multiple cell surface molecules (CD10, CD11b, CD11c, CD13, CD14, CD15, CD16, CD34, CD45, CD56, CD64 and HLA-DR) in PB neutrophils and monocytes from a series of 14 adult LR-MDS patients versus 14 normal individuals. Results: Peripheral blood neutrophils from patients with LR-MDS frequently had low forward scatter (FSC) and side scatter (SSC) values and low levels of CD11b, CD11c, CD10, CD16, CD13 and CD45 expression, in that order, as compared to normal neutrophils. In addition, patients with LR-MDS commonly display a higher fraction of CD14+CD56+ and a lower fraction of CD14+CD16+ monocytes in the PB. Based on these results, we proposed an immunophenotyping score based on which PB samples from patients with LR-MDS could be distinguished from normal PB samples with a sensitivity 93% and a specificity of 100%. In addition, we used this score to construct the MDS Thermometer, a screening tool for detection and monitoring of MDS in the PB in clinical practice. Conclusions: Peripheral blood neutrophil and monocyte immunophenotyping provide useful information for the diagnosis of LR-MDS, as a complement to cytomorphology. If validated by subsequent studies in larger series of MDS patients and extended to non-MDS patients with cytopenias, our findings may improve the diagnostic assessment and avoid invasive procedures in selected groups of MDS patients.
- RECOMENDAÇÕES PARA O DIAGNÓSTICO, TRATAMENTO E MONITORIZAÇÃO DA LEUCEMIA MIELÓIDE CRÓNICA.Publication . Almeida, A.; Castro, I.; Coutinho, J.; Guerra, L.; Marques, H.; Pereira, A.