RESUMO
In the past decade, patients with newly diagnosed diffuse large B-cell lymphoma (DLBCL) were treated with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone) therapy. Standard treatment is now changing as a result of deeper understanding of underlying biologic differences of such lymphomas. One of the most powerful predictors of an adverse outcome on R-CHOP therapy is the presence of a MYC gene rearrangement (MYC+ lymphoma). Determination of MYC gene rearrangement by FISH (fluorescent in situ hybridisation) has recently become a standard diagnostic procedure. In this paper, an overview of current literature on MYC function and MYC+ lymphoma patient outcome is presented. Furthermore, we present 26 patients from our tertiary referral centre who were diagnosed with MYC+ lymphoma between 2009 and 2014. In our patient series, we confirm the dismal prognosis of MYC+ lymphoma patients. Intensification of classical chemotherapy does not lead to better overall survival, justifying new treatment modalities. First line therapy should be more specifically targeted against MYC and the genes and proteins that are deregulated by MYC. To this end, the first clinical trial in which MYC+ patients will be offered targeted treatment has recently been launched.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biomarcadores Tumorais/genética , Rearranjo Gênico , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Proteínas Proto-Oncogênicas c-myc/genética , Adulto , Idoso , Feminino , Predisposição Genética para Doença , Humanos , Hibridização in Situ Fluorescente , Linfoma Difuso de Grandes Células B/genética , Linfoma Difuso de Grandes Células B/mortalidade , Linfoma Difuso de Grandes Células B/patologia , Masculino , Pessoa de Meia-Idade , Terapia de Alvo Molecular , Seleção de Pacientes , Fenótipo , Medicina de Precisão , Valor Preditivo dos Testes , Análise de Sobrevida , Resultado do TratamentoRESUMO
The gastrointestinal tract is the most common extranodal site of non-Hodgkin lymphoma. Certain lymphomas have a predilection for the gastrointestinal tract, including extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue, mantle cell lymphoma, natural killer/T-cell lymphoma, and enteropathy-associated T-cell lymphoma. Follicular lymphoma may also be primary to the gastrointestinal tract. In addition to diagnosing neoplastic conditions, it is important to differentiate lymphomas from atypical reactive proliferations. Recent research relevant to non-Hodgkin lymphomas involving this location is reviewed with an emphasis on novel and evolving areas of classification.