Morel Alexandre, Brière Josette, Lamant Laurence, Loschi Michaël, Haioun Corinne, Delarue Richard, Tournilhac Olivier, Bachy Emmanuel, Sonet Anne, Amorim Sandy, Laurent Camille, Gaulard Philippe, Tilly Hervé, Sibon David
Hematology Department, Necker University Hospital, Greater Paris University Hospitals (AP-HP), Paris Descartes University-Sorbonne Paris Cité, Imagine Institute, Paris, France.
Pathology Department, Necker University Hospital, AP-HP, Paris Descartes University-Sorbonne Paris Cité, Imagine Institute, Paris, France.
Eur J Cancer. 2017 Sep;83:146-153. doi: 10.1016/j.ejca.2017.06.026. Epub 2017 Jul 20.
Long-term outcomes of adults with first-relapsed/refractory (R/R) systemic anaplastic large-cell lymphoma (ALCL) are not definitively established and should be evaluated.
We previously published the long-term outcomes of adults with ALCL initially treated with polychemotherapy in LYmphoma Study Association (LYSA) prospective clinical trials conducted during the pre-brentuximab vedotin era. Herein, we report the long-term outcomes of those patients after the first-relapsed/refractory (R/R) events.
Among the 138 (64 (anaplastic lymphoma kinase (ALK(+)) and 74 ALK(-) ALCL) adults initially treated in clinical trials, 40 (14 ALK(+) and 26 ALK(-)) first-R/R ALCL patients and their long-term outcomes were analysed. Median follow-up from the first-R/R events was 12.5 years. For ALK(+) and ALK(-) patients, respectively, median [range] findings were as follows: age at first-R/R event: 35 [19-76] and 61 [34-81] years; time between inclusion in first-line clinical trials and first-R/R events was 6 [1.5-34] and 11.1 [1-67] months (P = 0.36); with median (95% confidence interval) progression-free survival after the first-R/R events: 3.8 (0.7-14.8) and 5.3 (2.4-8.4) months (P = 0.39); and overall survival: 13.6 (0.7-89) and 8.1 (3.3-25) months (P = 0.96). ALCL was the main cause of death.
Most adults with first-R/R ALCL have poor outcomes, with no significant differences between patients with ALK(+) or ALK(-) disease. These results could be used as reference for the evaluation of new drugs to treat R/R ALCL.
初治复发/难治性（R/R）系统性间变性大细胞淋巴瘤（ALCL）成人患者的长期预后尚未明确确定，需要进行评估。
我们之前在淋巴瘤研究协会（LYSA）开展的前瞻性临床试验中公布了初治接受多药化疗的成人ALCL患者的长期预后，这些试验是在本妥昔单抗时代之前进行的。在此，我们报告这些患者首次复发/难治（R/R）事件后的长期预后。
在最初纳入临床试验的138例成人患者（64例间变性淋巴瘤激酶（ALK）阳性和74例ALK阴性ALCL）中，分析了40例首次复发/难治性ALCL患者及其长期预后。首次复发/难治事件后的中位随访时间为12.5年。对于ALK阳性和ALK阴性患者，中位[范围]结果如下：首次复发/难治事件时的年龄：35[19 - 76]岁和61[34 - 81]岁；从纳入一线临床试验到首次复发/难治事件的时间为6[1.5 - 34]个月和11.1[1 - 67]个月（P = 0.36）；首次复发/难治事件后的无进展生存期（95%置信区间）中位数分别为：3.8（从0.7至14.8）个月和5.3（2.4至8.4）个月（P = 0.39）；总生存期分别为：13.6（0.7至89）个月和8.1（3.3至25）个月（P = 0.96）。ALCL是主要死因。
大多数首次复发/难治性ALCL成人患者预后较差，ALK阳性或ALK阴性疾病患者之间无显著差异。这些结果可作为评估治疗复发/难治性ALCL新药的参考。
