Division of Hematology and Bone Marrow Transplantation, Fondazione IRCCS Istituto Nazionale dei Tumori, University of Milano, Milan.
Unit of Hematology, Hospital C. Massaia, Asti.
Ann Oncol. 2014 Dec;25(12):2339-2350. doi: 10.1093/annonc/mdu152. Epub 2014 Apr 9.
In order to promote widespread adoption of appropriate clinical practice, the Italian Society of Hematology (SIE), and the affiliate societies SIES (Italian Society of Experimental Hematology) and GITMO (Italian Group for Bone Marrow Transplantation) established to produce guidelines in the most relevant hematological areas. In this article, we report the recommendations for management of T/NK-cell lymphomas, excluding mature T-cell leukaemias.
By using the Grades of Recommendations, Assessment, Development and Evaluation (GRADE) system, we produced evidence-based recommendations for the key clinical questions that needed to be addressed by a critical appraisal of evidence. The consensus methodology was applied to evidence-orphan issues.
Six courses of cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) or cyclophosphamide, doxorubicin, vincristine, etoposide and prednisone (CHOEP) chemotherapy were recommended for first-line therapy of patients with nodal, intestinal or hepatosplenic T-cell lymphomas (evidence: low; recommendation: do, weak). Except for ALK+ anaplastic large-cell lymphoma and elderly unfit patients, consolidation with high-dose chemotherapy was recommended (evidence: low; recommendation: do, weak). 50 Gy radiotherapy was the recommended first-line therapy for localized extranodal T/NK-cell lymphoma nasal type (evidence: low; recommendation: do, strong), while l-asparaginase-containing chemotherapy regimens were recommended for patients with systemic disease (evidence: very low; recommendation: do, strong).
In adult T/NK-cell lymphomas, GRADE methodology was applicable to a limited number of key therapeutic issues. For the remaining key issues, due to lack of appraisable evidence, recommendations was based on consensus methodology.
为了促进适当临床实践的广泛采用，意大利血液学学会（SIE）及其附属学会 SIES（意大利实验血液学学会）和 GITMO（意大利骨髓移植组）成立，以制定最相关的血液学领域的指南。在本文中，我们报告了管理 T/NK 细胞淋巴瘤的建议，不包括成熟 T 细胞白血病。
我们使用分级评估、制定与评价（GRADE）系统，通过对证据进行批判性评估，为需要解决的关键临床问题制定了基于证据的建议。共识方法适用于证据孤儿问题。
建议对结内、肠或肝脾 T 细胞淋巴瘤患者进行 6 个疗程的环磷酰胺、多柔比星、长春新碱和泼尼松（CHOP）或环磷酰胺、多柔比星、长春新碱、依托泊苷和泼尼松（CHOEP）化疗作为一线治疗（证据：低；推荐：做，弱）。除了 ALK+间变性大细胞淋巴瘤和老年不适患者外，建议进行大剂量化疗巩固治疗（证据：低；推荐：做，弱）。50 Gy 放疗是局限性结外 T/NK 细胞淋巴瘤鼻型的一线治疗推荐（证据：低；推荐：做，强），而含 l-天冬酰胺酶的化疗方案推荐用于全身疾病患者（证据：非常低；推荐：做，强）。
在成人 T/NK 细胞淋巴瘤中，GRADE 方法适用于少数关键治疗问题。对于其余的关键问题，由于缺乏可评估的证据，建议基于共识方法。
