P1208: CHARACTERISTICS AND CLINICAL OUTCOMES OF PATIENTS WITH ALK-POSITIVE ANAPLASTIC LARGE CELL LYMPHOMA: REPORT FROM THE PROSPECTIVE INTERNATIONAL T-CELL LYMPHOMA PROJECT

• DOI: 10.1097/01.hs9.0000847696.30063.c7
• Publication Date: 2022-06-23T14:22:35Z
• Authors: C. Chiattone; M. Civallero; T. Fischer; E. Miranda; M. Manni; N. P. C. Zing; S. A. Pileri; S. Montoto; S. M. Horwitz; M. E. Cabrera; C. A. De Souza; A. Nagler; S. Luminari; A. J. M. Ferreri; K. R. Carson; A. Re; L. Rigacci; L. Nassi; M. Federico; G. Inghirami
• Keywords: Anaplastic large-cell lymphoma; International Prognostic Index

Background: Anaplastic large cell lymphoma kinase-positive (ALCL, ALK+) is a distinct lymphoid neoplasm whose malignant cells express CD30 (TNFR8), and carrying translocations of the anaplastic kinase. Downstream ALK signaling cascades modulate both anti apoptotic signals growth patterns fostering proliferation promoting an aggressive clinical behavior. T-cell Lymphoma Project (TCP) largest prospective multicenter cohort study conducted in newly diagnosed PTCL patients. Herein, we report analysis demographics, initial characteristics, treatment patterns, outcomes ALCL, ALK+ patients enrolled into TCP database. Aims: We Methods: Seventy-four institutions from 14 countries North America, South Europe, Asia collected data on treated at respective centers between September 2006 February 2018. One hundred thirty-one (8.4% total cohort) were confirmed to have - kinase positive ALK+), among 1553 Results: Patients’ median age was 39 years (18-84). Sixty-five (66%) had advanced-stage disease, although majority (45 patients, 54%) low-risk International Prognostic Index score (0-1). Of 81 with chemotherapy, 97% received anthracycline-containing regimens. The overall response rate 81%, 69 (70%) achieving complete remission. OS PFS estimated 3 77% (95% CI 55-99%) 68% 46-90%), respectively, 5 very similar 62-92%) 64% 34-94%). Multivariate for results advanced stage (HR 4.72 95%CI: 1.43-23.9, P=0.015), elevated LDH 4.85 1.73-13.60, P=0.014) ECOG-PS≥2 5.25 1.68-16.4, P=0.024). Whereas resulted 3.77 1.98-14.17, ECOG-PS ≥2 4.59 1.46-14.39, P=0.004). Summary/Conclusion: Considering low incidence heterogeneity, diagnostic algorithms therapeutic guidelines remain challenging. Indeed, CHOP-like regimens ALCL ALK+‘s lead favorable responses, ~30% need second-line therapy (refractory or relapsed patients). Developing more specific/effective first/second-line protocols achieve long-term rates/eradication preserve safety profiles highly desirable. anticipate that only international-based studies will improve our collective knowledge ameliorate outcomes.