Tamara Intermesoli1, Alessandra Weber1, Matteo Leoncin2, Luca Frison2, Cristina Skert2, Renato Bassan3. 1. Hematology and Bone Marrow Transplant Unit, Azienda Socio Sanitaria Territoriale (ASST), Ospedale Papa Giovanni XXIII, 24127, Bergamo, Italy. 2. Hematology Unit, Azienda Ulss3 Serenissima, Ospedale dell'Angelo, Venezia-Mestre, 30174, Venice, Italy. 3. Hematology Unit, Azienda Ulss3 Serenissima, Ospedale dell'Angelo, Venezia-Mestre, 30174, Venice, Italy. renato.bassan@aulss3.veneto.it.
Abstract
PURPOSE OF REVIEW: Lymphoblastic 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 FINDINGS: T-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. Although 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.
PURPOSE OF REVIEW: Lymphoblastic 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 FINDINGS: T-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. Although 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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