医学
淋巴瘤
淋巴母细胞淋巴瘤
美罗华
内科学
肿瘤科
化疗
作者
Tamara Intermesoli,Alessandra Weber,Matteo Leoncin,Luca Frison,Cristina Skert,Renato Bassan
标识
DOI:10.1007/s11912-021-01168-x
摘要
Purpose of ReviewLymphoblastic lymphoma (LBL) is a rare, highly aggressive non-Hodgkin lymphoma variant virtually indistinguishable from acute lymphoblastic leukemia (ALL). We review the advancements in diagnostics, staging, treatment, and response assessment.Recent FindingsT-LBL displays a mediastinal mass with pleuro-pericardic effusions as key distinctive features and is far more frequent than B-LBL. LBL is exquisitely sensitive to ALL-type chemotherapy, achieving cure rates in the order of 70% in adults and even more in children. Positron-emission tomography, genetic risk classifications, and minimal disseminated/residual disease assays are increasingly used to detect occult sites of involvement and predict treatment outcome. Stem cell transplantation is effective and should be considered for very high-risk subsets and/or at salvage.SummaryAlthough curable in the majority of patients, about 25–30% of adults with LBL patients experience resistance or relapse following first-line therapy. It is essential to identify these cases early on and to explore new modalities of precision medicine with targeted agents.
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