Title: ANAPLASTIC LARGE CELL LYMPHOMA, ALK‐NEGATIVE: ANALYSIS OF 235 CASES COLLECTED BY THE T‐CELL PROJECT
Abstract: Introduction: Anaplastic Large Cell Lymphoma, ALK-Negative(ALCL, ALK-) is an aggressive lymphoid neoplasm of T/null-cell lineage with strong expression of CD30, which represent 5.5% of all PTCL and NKTCL, with a unique ethnic and geographical distribution. ALCL, ALK- displays a poorer outcome compared to ALK-positive, with a 5-year OS of 49% and 70%, respectively. This study aims to provide accurate and update information on prognostic factors, therapy, and outcomes of ALK- ALCL registered in the T-Cell Project. Methods: Eligible patients (pts) with the first diagnosis of aggressive, mature PTCLs were prospectively registered at a dedicated website and baseline demographic, clinical and laboratory data, as well as therapy details and outcome data were collected. Results: From Sept 2006 to Feb 2018, a diagnosis of ALCL, ALK- was reported in 235 cases out of 1553 validated cases registered by 77 sites from 14 countries worldwide. The median age at diagnosis was 54 (range, 18-89) years with male predominance (61%). Stage III-IV disease was identified in 71% pts, bulky and bone marrow involvement were uncommon. Therapy was administered according to treating physician's standard practice. Out of 205 patients for which we have available data about treatment, in 9% were addressed to consolidating post-transplant chemotherapy. The overall response rate was 77% with 57% of patients achieving complete remission. After a median follow-up of 55 (95% CI, 36-75) months, the PFS was 41 (95% CI, 17-62) months, the 3-year and 5-year PFS rates were 47% and 39%, respectively. The median OS was 55 (95% CI, 36-75) months, 3-year and 5-year OS rates were 60% (95CI 48-72) and 49% (95CI35-59), respectively. International Prognostic Index (IPI) and the Prognostic Index for T-cell Lymphomas (PIT) were both predictive of clinical outcomes. Treatment with anthracycline and etoposide vs. anthracycline-only containing regimens was associated with a better OS( 3 and 5-year OS 56% and 44% vs 76% and 69%, respectively, (P = 0.05), but not PFS 3 and 5 year PFS 47% (95CI 32-59) and 39 % (95CI 29-48) vs. 65% (95CI 32-87) and 50% (95CI 36-89) respectively (P = 0.186). In the multivariate analysis the presence of B symptoms (P = 0.008), elevated LDH rate (P = 0.001), ECOG-PS >2 (P = 0.001) and PTL<150x109(P = 0.05), were significant for OS. The presence of B symptoms (p = 0.02), elevated LDH (P = 0.001) and ECOG-PS>2 (P = 0.001) maintained their adverse prognostic value for PFS. Conclusions: ALCL, ALK- remains a difficult T cell lymphoma to be cured. Combinations regimens that include both etoposide and anthracycline might improve outcomes. In this large prospective cohort study, we observed an outcome comparable with a previous report from the retrospective International Lymphoma Project. The study underscores the urgent need for designing new treatment platforms for ALCL, ALK-, and establishes a benchmark for future prospective clinical trials. Keywords: anaplastic large cell lymphoma (ALCL); prognostic indices; T-cell lymphoma (TCL). Disclosures: Horwitz, S: Consultant Advisory Role: Affimed, Angimmune, Beigene, Corvus, Innate Pharma, Kura, Merck, Miragen, Mundipharma, Portola, Syros Pharmaceutical, ADCT Therapeutics, Aileron, Forty-Seven Infinity/Verastem Kyowa-Hakka-Kirin, Millennium / Takeda, Seattle Genetics; Research Funding: Celgene, Trillium, ADCT Therapeutics, Aileron, Forty-Seven Infinity/Verastem Kyowa-Hakka-Kirin, Millennium / Takeda, Seattle Genetics. Spina, M: Consultant Advisory Role: Menarini, Mundipharma, Teva, GILEAD, Janssen-Cilag, CTI, Servier, Sandoz, Novartis, Pfizer; Honoraria: Mundipharma, Teva, GILEAD, Janssen-Cilag, CTI, Servier, Celgene.
Publication Year: 2019
Publication Date: 2019-06-01
Language: en
Type: article
Indexed In: ['crossref']
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Cited By Count: 1
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