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Lymphoblastic lymphoma: an updated review on biology, diagnosis, and treatment
Author(s) -
Bassan Renato,
Maino Elena,
Cortelazzo Sergio
Publication year - 2016
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/ejh.12722
Subject(s) - lymphoblastic lymphoma , medicine , immunophenotyping , minimal residual disease , lymphoma , radiation therapy , blinatumomab , oncology , bone marrow , positron emission tomography , mediastinum , leukemia , lymphoblastic leukemia , pediatrics , immunology , radiology , t cell , antigen , immune system
Lymphoblastic lymphoma is a rare aggressive neoplasm of T‐/B‐precursors resembling acute lymphoblastic leukemia, with no or limited bone marrow involvement (<25%), that develops more frequently in children and young adults and is typically characterized by a grossly enlarged mediastinum, and whose diagnostic hallmark is the expression of a T‐/B‐precursor cell immunophenotype, the T‐cell subset accounting for 90% of all cases. The adoption of pediatric‐derived, intensive lymphoblastic leukemia‐like protocols led to significantly improved results, with survival rates of about 70% and 90% in adults and children, respectively. Adequate central nervous system prophylaxis and mediastinal irradiation contributed to the therapeutic success; however, the role of radiation therapy is debated due to toxicity concerns and the excellent results obtained with radiation‐free programs especially in pediatric patients. With these modern schedules, localized radiotherapy and/or hematopoietic stem cell transplants could be generally omitted, and considered only for high‐risk patients identified through postinduction computed tomography/positron‐emission tomography scans, minimal residual disease analysis, and new genetics and genomics. New clinical studies will have to confirm the value of these assays for risk‐oriented therapy, while further therapeutic progress is expected from the introduction of new drugs and targeting agents.