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Lymphoblastic Lymphoma: an Updated Review on Biology, Diagnosis, and Treatment

European journal of haematology(2016)

Osped Angelo | Clin Humanitas Gavazzeni

Cited 93|Views1
Abstract
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.
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lymphoblastic lymphoma,biology,diagnosis,risk factors,treatment
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要点】:本文综述了淋巴母细胞性淋巴瘤的生物学特征、诊断方法及治疗进展,特别强调了对儿童和年轻成人患者的治疗策略及现代治疗方案的创新应用。

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方法】:文章通过文献回顾的方式,总结了淋巴母细胞性淋巴瘤的生物学、诊断和治疗方面的研究成果**。

实验】:本文未涉及具体实验,但提到了采用儿科来源的强化治疗方案,以及通过诱导后CT/PET扫描、微小残留病分析和新的遗传与基因组学方法来识别高风险患者,并使用这些方法指导风险导向治疗**。