Department of Medicine A, University Hospital Münster, Münster, Germany.
Blood. 2018 Jul 19;132(3):245-253. doi: 10.1182/blood-2018-01-791335. Epub 2018 Apr 26.
Mature T- and natural killer (NK)-cell neoplasms comprise a group of morphologically, immunophenotypically, molecularly, and clinically heterogeneous disorders with generally unfavorable outcome. Results of first-line chemotherapy are unsatisfactory for the most common T-cell lymphomas (peripheral T-cell lymphoma, not otherwise specified; angioimmunoblastic T-cell lymphoma; anaplastic large cell lymphomas; anaplastic lymphoma tyrosine kinase-negative) as well as for many other entities. High-dose therapy followed by autologous hematopoietic stem cell transplantation (HSCT) is widely recommended for consolidation after a complete or partial remission is achieved. However, about one-third of patients never reach transplantation because of early relapse or refractoriness. Targeted therapies have recently been developed; combinations with chemotherapy may improve outcomes, but long-term results from prospective studies are largely missing. In this situation, allogeneic HSCT remains a valuable treatment option inducing long-lived remissions in about 30% to 50% of patients with relapsed and refractory T-cell lymphoma able to proceed to transplantation. Results of allogeneic transplantation for consolidation in first remission are less defined and its indications remain controversial. With growing evidence that haploidentical HSCT also works in lymphoma, more patients can be brought to transplantation. Decreasing the morbidity and mortality of allogeneic transplantation is a continuous challenge. Integrating new drugs into transplant concepts and setting up prospective studies involving allogeneic transplantation remain unmet needs that warrant urgent study in a group of disorders in which classical chemotherapy and new drugs have generated results, which are far from optimal until today.
成熟 T 细胞和自然杀伤(NK)细胞肿瘤包括一组形态、免疫表型、分子和临床表现均具有异质性的疾病,总体预后较差。对于最常见的 T 细胞淋巴瘤(外周 T 细胞淋巴瘤,未另作说明;血管免疫母细胞性 T 细胞淋巴瘤;间变大细胞淋巴瘤;间变性淋巴瘤激酶阴性)以及许多其他实体瘤,一线化疗的效果并不令人满意。对于达到完全或部分缓解的患者,通常建议进行高剂量化疗后自体造血干细胞移植(HSCT)巩固治疗。然而,约三分之一的患者由于早期复发或耐药而无法进行移植。最近已经开发了靶向治疗方法;与化疗联合可能会改善预后,但前瞻性研究的长期结果基本缺失。在这种情况下,异基因 HSCT 仍然是一种有价值的治疗选择,可使约 30%至 50%能够进行移植的复发和难治性 T 细胞淋巴瘤患者获得长期缓解。在首次缓解期进行巩固性异基因 HSCT 的结果不太明确,其适应证仍存在争议。随着越来越多的证据表明单倍体 HSCT 也适用于淋巴瘤,更多的患者可以进行移植。降低异基因移植的发病率和死亡率是一个持续的挑战。将新药纳入移植方案并建立包括异基因移植的前瞻性研究仍然是未满足的需求,对于一组疾病来说,这是一个紧迫的研究课题,这些疾病的经典化疗和新药已经取得了一定的效果,但迄今为止仍远不理想。