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Leucemia linfocítica crónica: sintomas, diagnóstico e abordagens terapêuticas
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A Leucemia Linfocítica Crónica (LLC) é a forma de leucemia com maior taxa de diagnóstico na Europa Ocidental e América do Norte, constituindo o tipo de leucemia mais comum em adultos. Classificada como uma desordem linfoproliferativa, resulta da acumulação progressiva no sangue periférico, medula óssea, nódulos linfáticos e baço, de linfócitos B maduros com imunofenótipo peculiar.
O seu curso clínico é caracterizado pela sua heterogeneidade. Enquanto que, na maioria dos doentes, a LLC se apresenta sem sintomatologia, necessitando apenas de uma vigilância cuidadosa durante muitos anos, noutros casos, a doença manifesta-se de forma agressiva, e o tratamento é necessário logo após o seu diagnóstico. Este é estabelecido através da contagem absoluta de linfócitos igual ou superior a 5000 células/mm3, e por um fenótipo característico, que resulta da combinação entre o antigénio CD5 (presente na linhagem T) com os marcadores dos linfócitos B típicos - CD19, CD20 e CD23.
O estadiamento da doença é baseado nas classificações de Rai e Binet, contudo novos marcadores de prognóstico têm sido descobertos, permitindo uma melhoria na estratificação dos doentes.
Apesar da LLC continuar a ser uma doença incurável, ao longo da última década, têm sido alcançados avanços importantes no conhecimento da sua biologia e no seu arsenal terapêutico. A imunoquimioterapia é considerada a primeira-linha de tratamento dos doentes com LLC, todavia, novos agentes direcionados como os inibidores da tirosina cinase de Bruton (BTK), inibidores da fosfatidilinositol-3-cinase (PI3K) e inibidores da BCL-2, têm sido estudados como tratamento de primeira-linha e doença refratária/recidiva, em monoterapia e em terapias combinadas, apresentando-se como propostas promissoras com um perfil de segurança favorável.
Chronic Lymphocytic Leukemia (CLL) is the leukemia form with higher rates of diagnosis in Western Europe and North America, representing the most common type of leukemia in adults. Classified as a lymphoproliferative disorder, results from the progressive accumulation in peripheral blood, bone marrow, lymph nodes and spleen of mature B lymphocytes with a peculiar immunophenotype. Its clinical course is characterized by its heterogenicity. While in most patients, CLL presents no symptoms, requiring only a careful monitoring for many years, in other cases, the disease manifests itself aggressively, and treatment is required immediately after diagnosis. This is established by absolute lymphocyte count equal or higher than 5000 cells/mm3 and by a distinctive phenotype that results from the combination of CD5 antigen (present in the T lineage) with typical B lymphocytes markers - CD19, CD20 and CD23. The staging of the disease is based on Rai and Binet ratings, however new prognostic markers have been discovered, allowing an improved stratification of the patients. Although CLL remains an incurable disease, important advances have been achieved over the last decade in the knowledge of its biology and therapeutic arsenal. The immunochemotherapy is considered first-line treatment of patients with CLL. However, new target agents as tyrosine kinase inhibitors of Bruton (BTK), phosphatidylinositol-3 kinase inhibitors (PI3K) and BCL-2 inhibitors have been studied as first-line and refractory/relapse therapy in monotherapy and in combination therapy, presenting themselves as promising proposals with a favorable safety profile.
Chronic Lymphocytic Leukemia (CLL) is the leukemia form with higher rates of diagnosis in Western Europe and North America, representing the most common type of leukemia in adults. Classified as a lymphoproliferative disorder, results from the progressive accumulation in peripheral blood, bone marrow, lymph nodes and spleen of mature B lymphocytes with a peculiar immunophenotype. Its clinical course is characterized by its heterogenicity. While in most patients, CLL presents no symptoms, requiring only a careful monitoring for many years, in other cases, the disease manifests itself aggressively, and treatment is required immediately after diagnosis. This is established by absolute lymphocyte count equal or higher than 5000 cells/mm3 and by a distinctive phenotype that results from the combination of CD5 antigen (present in the T lineage) with typical B lymphocytes markers - CD19, CD20 and CD23. The staging of the disease is based on Rai and Binet ratings, however new prognostic markers have been discovered, allowing an improved stratification of the patients. Although CLL remains an incurable disease, important advances have been achieved over the last decade in the knowledge of its biology and therapeutic arsenal. The immunochemotherapy is considered first-line treatment of patients with CLL. However, new target agents as tyrosine kinase inhibitors of Bruton (BTK), phosphatidylinositol-3 kinase inhibitors (PI3K) and BCL-2 inhibitors have been studied as first-line and refractory/relapse therapy in monotherapy and in combination therapy, presenting themselves as promising proposals with a favorable safety profile.
Descrição
Trabalho Final de Mestrado Integrado, Ciências Farmacêuticas, Universidade de Lisboa, Faculdade de Farmácia, 2016
Palavras-chave
Leucemia linfocítica crónica Linfócitos B Sintomatologia Diagnóstico Marcadores de prognóstico Tratamento Mestrado Integrado - 2016