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Artigo em Inglês | MEDLINE | ID: mdl-32713742


Paroxysmal nocturnal hemoglobinuria is a chronic, multi-systemic, progressive and life-threatening disease characterized by intravascular hemolysis, thrombotic events, serious infections and bone marrow failure. Paroxysmal nocturnal hemoglobinuria results from the expansion of a clone of hematopoietic cells that due to an inactivating mutation of the X-linked gene PIG-A are deficient in glycosylphosphatidylinositol-linked proteins. Early diagnosis, using flow cytometry performed on peripheral blood, the gold standard test to confirm the diagnosis of paroxysmal nocturnal hemoglobinuria, is essential for improved patient management and prognosis. The traditional therapy for paroxysmal nocturnal hemoglobinuria includes blood transfusion, anti-thrombosis prophylaxis or allogeneic bone marrow transplantation. The treatment that has recently become available is the complement blockade by the anti-C5 monoclonal antibody eculizumab. In this consensus, we are aiming to review the diagnosis and treatment of the paroxysmal nocturnal hemoglobinuria patients, as well as the early recognition of its systemic complications. These procedures express the opinions of experts and have been based on the best available evidence and international guidelines, with the purpose of increasing benefits and reducing harm to patients.

J Glob Oncol ; 5: 1-19, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31774711


PURPOSE: Limited information is available on multiple myeloma (MM), chronic lymphocytic leukemia (CLL), and non-Hodgkin lymphoma (NHL) management in Latin America. The primary objective of the Hemato-Oncology Latin America (HOLA) study was to describe patient characteristics and treatment patterns of Latin American patients with MM, CLL, and NHL. METHODS: This study was a multicenter, retrospective, medical chart review of patients with MM, CLL, and NHL in Latin America identified between January 1, 2006, and December 31, 2015. Included were adults with at least 1 year of follow-up (except in cases of death within 1 year of diagnosis) treated at 30 oncology hospitals (Argentina, 5; Brazil, 9; Chile, 1; Colombia, 5; Mexico, 6; Panama/Guatemala, 4). RESULTS: Of 5,140 patients, 2,967 (57.7%) had NHL, 1,518 (29.5%) MM, and 655 (12.7%) CLL. Median follow-up was 2.2 years for MM, 3.0 years for CLL, and 2.2 years for NHL, and approximately 26% died during the study observation period. Most patients had at least one comorbidity at diagnosis. The most frequent induction regimen was thalidomide-based chemotherapy for MM and chlorambucil with or without prednisone for CLL. Most patients with NHL had diffuse large B-cell lymphoma (DLBCL; 49.1%) or follicular lymphoma (FL; 19.5%). The majority of patients with DLBCL or FL received rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone. CONCLUSION: The HOLA study generated an unprecedented level of high-quality, real-world evidence on characteristics and treatment patterns of patients with hematologic malignancies. Regional disparities in patient characteristics may reflect differences in ethnoracial identity and level of access to care. These data provide needed real-world evidence to understand the disease landscape in Latin America and may be used to inform clinical and health policy decision making.

Leucemia Linfocítica Crônica de Células B/epidemiologia , Linfoma não Hodgkin/epidemiologia , Mieloma Múltiplo/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Humanos , América Latina/epidemiologia , Pessoa de Meia-Idade , Sistema de Registros , Adulto Jovem
Rev. Soc. Cardiol. Estado de Säo Paulo ; 19(4): 562-571, out.-dez. 2009. tab
Artigo em Português | LILACS | ID: lil-559943


Tromboembolismo venoso é um evento potencialmente fatal, que complica a evolução de 20 por cento dos pacientes com neoplasia, causando impacto significativo na qualidade de vida e nos desfechos clínicos desses pacientes. A fisiopatologia da associação entre trombose e câncer é complexa. As neoplasias estão associadas a estado de hipercoagulabilidade secundário à liberação de citocinas inflamatórias, ativação do sistema de coagulação, expressão de proteínas hemostáticas nas células tumorais, inibição de anticoagulantes naturais e alteração no processo fibrinolítico. Os fatores de risco associados ao tromboembolismo venoso podem ser relacionados ao paciente, à doença ou às intervenções terapêuticas. O uso profilático de heparinas e fondaparinux está indicado em pacientes selecionados de acordo com o tipo de neoplasia e da terapia utilizada. O tratamento dos pacientes oncológicos com tromboembolismo venoso é desafiador: as complicações associadas ao uso de anticoagulantes são significativamente maiores, podem interferir com o tratamento antineoplásico e têm impacto negativo na qualidade de vida.

Venous thromboembolism is a serious and potentially fatal disorder, which complicates the course of 20% of cancer patients, and has a significant impact on the quality of life and clinical outcomes of these patients. The pathophysiology of the association between thrombosis and cancer is complex. Malignancy is associated with a baseline hypercoagulable state secondary to the release of inflammatory cytokines, activation of the clotting system, expression of hemostatic proteins on tumor cells, inhibition of natural anticoagulants and impaired fibrinolysis. Several risk factors have been identified as contributing to venous thromboembolism and may be related to the patient characteristics, to the disease, and to the therapeutic interventions. The use of heparins and fondaparinux is indicated for selected cancer patients according to the types of malignancies and treatments. The treatment of venous thromboembolism in patients with cancer is challenging: the complications associated with the use of anticoagulants are significantly higher, may interfere with anticancer therapy and have a negative impact on quality of life.

Humanos , Masculino , Feminino , Idoso , Coagulação Sanguínea , Neoplasias/complicações , Tromboembolia Venosa/complicações , Tromboembolia Venosa/fisiopatologia , Qualidade de Vida , Fatores de Risco