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Evolução do mieloma múltiplo sob tratamento com os novos fármacos : a propósito de um caso clínico
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O mieloma múltiplo é uma neoplasia maligna que resulta da proliferação de plasmócitos clonais. É uma doença que se desenvolve ao longo de várias fases e que se manifesta com hipercalcemia, insuficiência renal, anemia e lesões ósseas.
Nos últimos 20 anos, o tratamento do mieloma tem vindo a alterar-se com a introdução de novos fármacos na prática clínica. O inibidor do proteossoma bortezomib e os agentes imunomoduladores talidomida e lenalidomida revolucionaram o tratamento dos doentes recém-diagnosticados, melhorando significativamente o prognóstico, mas na generalidade dos casos ocorre recaída após tratamento inicial. A par destes, estão a ser introduzidos no tratamento das recaídas e principalmente da doença refratária, fármacos ainda mais recentes. Estes fármacos, desenvolvidos com base nos conhecimentos obtidos pela intensa investigação da arquitetura genética do mieloma e da sua relação com a medula óssea, que atualmente se observa, têm prolongado ainda mais a sobrevida dos doentes. Apesar disso, a doença invariavelmente evolui e nas suas fases mais tardias, pode perder a dependência da medula óssea e progredir para doença extra-medular, à qual se associa um mau prognóstico.
Neste trabalho, será apresentado um caso clínico de uma mulher de 72 anos, a quem foi diagnosticado mieloma múltiplo no contexto dum episódio de insuficiência renal aguda. Esta doente foi alvo de vários esquemas terapêuticos, durante aproximadamente 5 anos, acabando por sofrer progressão da doença sob a forma de plasmocitoma extra-medular.
Multiple myeloma is a malignancy that results from the clonal plasma cells proliferation. It’s a disease that develops during several stages and that emerges with hypercalcemia, renal failure, anemia and bone lesions. Over the last 20 years, the myeloma treatment has been changing with the introduction of new agents in clinical practice. Proteossoma inhibitor bortezomib and immunomodulatory agents thalidomide and lenalidomide revolutionized the treatment of newly diagnosed patients, improving significantly the prognosis. However, in most cases, relapse occurs after the initial treatment. In addition, more recent agents are being introduced in the relapses treatment and mainly in the refractory disease. These agents were developed based on the knowledge obtained by the intensive investigation of the genetic architecture of myeloma and its relationship with bone marrow, which is currently observed, have prolonged the survival of patients even more. Although, the disease invariably develops and in its later stages, it can lose the dependence of the bone marrow and progress to extra-medullary disease, which is associated with a poor prognosis. It will be presented the case of a 72-year-old-woman, to whom was diagnosed multiple myeloma in what concerns the investigation of a clinical presentation of acute renal failure. This patient was object of several therapeutic approaches during almost 5 years. Finally, the disease progressed into an extramedullary plasmacytoma.
Multiple myeloma is a malignancy that results from the clonal plasma cells proliferation. It’s a disease that develops during several stages and that emerges with hypercalcemia, renal failure, anemia and bone lesions. Over the last 20 years, the myeloma treatment has been changing with the introduction of new agents in clinical practice. Proteossoma inhibitor bortezomib and immunomodulatory agents thalidomide and lenalidomide revolutionized the treatment of newly diagnosed patients, improving significantly the prognosis. However, in most cases, relapse occurs after the initial treatment. In addition, more recent agents are being introduced in the relapses treatment and mainly in the refractory disease. These agents were developed based on the knowledge obtained by the intensive investigation of the genetic architecture of myeloma and its relationship with bone marrow, which is currently observed, have prolonged the survival of patients even more. Although, the disease invariably develops and in its later stages, it can lose the dependence of the bone marrow and progress to extra-medullary disease, which is associated with a poor prognosis. It will be presented the case of a 72-year-old-woman, to whom was diagnosed multiple myeloma in what concerns the investigation of a clinical presentation of acute renal failure. This patient was object of several therapeutic approaches during almost 5 years. Finally, the disease progressed into an extramedullary plasmacytoma.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2017
Palavras-chave
Mieloma múltiplo Plasmocitoma extra-medular Arquitetura genética Microambiente tumoral Hematologia