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ALK-negative anaplastic large cell lymphoma: features and outcomes of 235 patients from the International T-Cell Project. | LitMetric

AI Article Synopsis

  • Anaplastic lymphoma kinase-negative anaplastic large cell lymphoma (ALK- ALCL) is a type of aggressive cancer affecting T-cells, with a median diagnosis age of 54 and a majority of male patients (62%).
  • Out of 1,553 cases studied globally, 71% were at advanced stages (III-IV), and 85% received multiagent chemotherapy, showing initial response rates of 77% overall and 63% complete.
  • After a median follow-up of 52 months, progression-free survival was 41 months, and overall survival was 55 months, with treatments including anthracycline and etoposide yielding better overall survival outcomes.

Article Abstract

Anaplastic lymphoma kinase-negative anaplastic large cell lymphoma (ALK- ALCL) is an aggressive neoplasm of T-cell/null-cell lineage. The T-Cell Project is a global prospective cohort study that consecutively enrolled patients newly diagnosed with peripheral T-cell lymphoma, registered through a centralized computer database between September 2006 and February 2018. Of 1553 validated cases from 74 sites in 13 countries worldwide, 235 were reported as ALK- ALCL. The median age at diagnosis was 54 years (range, 18-89 years), with a male predominance (62%). Stage III to IV disease was identified in 71% of patients, bulky disease and bone marrow involvement were uncommon, and 66% of patients presented with a low (0-1) International Prognostic Index score. Of all treated patients, 85% received multiagent initial chemotherapy, and 8% were consolidated with autologous hematopoietic cell transplantation. The initial overall and complete response rates were 77% and 63%, respectively. After a median follow-up of 52 months (95% confidence interval [CI], 41-63), the median progression-free survival (PFS) and overall survival (OS) were 41 months (95% CI, 17-62) and 55 months (95% CI, 36-75), respectively. The 3- and 5-year PFS rates were 52% and 43%, and the 3- and 5-year OS rates were 60% and 49%. Treatments containing both anthracycline and etoposide were associated with superior OS (P = .05) but not PFS (P = .18). In this large prospective cohort study, outcomes comparable to those previously reported in the retrospective International Peripheral T-Cell Lymphoma Project were observed. The study underscores the need for introducing novel platforms for ALK- ALCL and establishes a benchmark for future clinical trials. This trial was registered at www.clinicaltrials.gov as #NCT01142674.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7876884PMC
http://dx.doi.org/10.1182/bloodadvances.2020001581DOI Listing

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