Lazzarotto Davide, Cerrano Marco, Papayannidis Cristina, Chiaretti Sabina, Mosna Federico, Fracchiolla Nicola, Zappasodi Patrizia, Imbergamo Silvia, Del Principe Maria Ilaria, Lunghi Monia, Lussana Federico, Piccini Matteo, Fumagalli Monica, Dargenio Michelina, Salutari Prassede, Forghieri Fabio, Da Molin Teresa Giulia, Bonifacio Massimiliano, Olivi Matteo, Giglio Fabio, Trappolini Silvia, Leoncin Matteo, Mule Antonino, Delia Mario, Pasciolla Crescenza, Grimaldi Francesco, Cambo Benedetta, Santoro Lidia, Guolo Fabio, Minetto Paola, Defina Marzia, Chiusolo Patrizia, Fanin Matteo, Mauro Endri, Aprile Lara, Mazzone Carla, Trastulli Fabio, Ciccone Maria, De Gobbi Marco, Cignetti Alessandro, De Bellis Eleonora, Mancini Valentina, Piciocchi Alfonso, Vignetti Marco, Marsili Giovanni, Starza Irene Della, Fanin Renato, Luppi Mario, Ferrara Felicetto, Pizzolo Giovanni, Bassan Renato, Foa Robin, Candoni Anna
Clinica Ematologica - Centro Trapianti e Terapie Cellulari, Azienda Sanitaria Universitaria Friuli Centrale, Udine, Italy.
S.C. Ematologia, AOU Citta della Salute e della Scienza - Presidio Molinette, Torino, Italy.
Haematologica. 2025 Jan 1;110(1):55-67. doi: 10.3324/haematol.2024.285638.
The introduction of pediatric-inspired regimens in adult Philadelphia-negative acute lymphoblastic leukemia (Ph- ALL) has significantly improved patients' prognosis. Within the Campus ALL network, we analyzed the outcome of adult Ph- ALL patients treated according to the GIMEMA LAL1913 protocol outside the clinical trial to compare the real-life data with the study results. We included 421 consecutive patients; median age 42 years. The complete remission (CR) rate after the first course of chemotherapy was 94%, and measurable residual disease (MRD) negativity after the third course was achieved in 72% of patients. The 3-year overall survival (OS) and disease-free survival (DFS) were 67% and 57%, respectively. In a multivariate analysis, MRD positivity negatively influenced DFS. In a time-dependent analysis including only very high-risk (VHR) and MRD positive cases, transplanted (hematopoietic stem cell transplantation [HSCT]) patients had a significantly better DFS than non-HSCT patients (P=0.0017). During induction, grade ≥2 pegaspargase-related hepato-toxicity was observed in 25% of patients (vs. 12% in the GIMEMA LAL1913 trial, P=0.0003). In this large, real-life cohort of Ph- ALL, we confirmed the very high CR rate and a superimposable OS and DFS compared to the GIMEMA LAL1913 clinical trial (CR rate after C1, 94% vs. 85%, P=0.0004; 3-year OS, 67% vs. 67%, P=0.94; 3-year DFS, 57% vs. 63%, P=0.17). HSCT confirms its important role in VHR and MRD-positive patients. The rate of pegaspargase-related toxicity was significantly higher in the real-life setting, emphasizing the importance of dose adjustment in the presence of risk factors to avoid excessive toxicity.
在成人费城染色体阴性急性淋巴细胞白血病(Ph-ALL)中引入儿童启发式治疗方案显著改善了患者的预后。在校园ALL网络中,我们分析了根据GIMEMA LAL1913方案在临床试验之外接受治疗的成人Ph-ALL患者的结局,以将真实数据与研究结果进行比较。我们纳入了421例连续患者;中位年龄42岁。第一个化疗疗程后的完全缓解(CR)率为94%,第三个疗程后72%的患者达到微小残留病(MRD)阴性。3年总生存期(OS)和无病生存期(DFS)分别为67%和57%。在多变量分析中,MRD阳性对DFS有负面影响。在仅包括极高危(VHR)和MRD阳性病例的时间依赖性分析中,接受移植(造血干细胞移植[HSCT])的患者DFS显著优于未接受HSCT的患者(P=0.0017)。在诱导期间,25%的患者观察到≥2级培门冬酶相关肝毒性(GIMEMA LAL1913试验中为12%,P=0.0003)。在这个大型的Ph-ALL真实队列中,我们证实了与GIMEMA LAL1913临床试验相比非常高的CR率以及可叠加的OS和DFS(C1后的CR率,94%对85%,P=0.0004;3年OS,67%对67%,P=0.94;3年DFS,57%对63%,P=0.17)。HSCT证实了其在VHR和MRD阳性患者中的重要作用。在真实环境中培门冬酶相关毒性发生率显著更高,强调了在存在危险因素时进行剂量调整以避免过度毒性的重要性。