ALK-negative anaplastic large cell lymphoma: Features and outcomes of 235 patients from the International T-Cell Project: Blood Advances

International T-cell Project Network

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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 5.05) but not PFS (P 5.18). 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 as #NCT01142674. © 2021 by The American Society of Hematology
Original languageEnglish
Pages (from-to)640-648
Number of pages8
JournalBlood Adv.
Issue number3
Publication statusPublished - 2021


  • anaplastic lymphoma kinase
  • anthracycline
  • etoposide
  • adult
  • aged
  • anaplastic large cell lymphoma
  • Article
  • autologous hematopoietic stem cell transplantation
  • cancer staging
  • cancer survival
  • clinical feature
  • clinical outcome
  • cohort analysis
  • controlled study
  • female
  • follow up
  • human
  • International Prognostic Index
  • major clinical study
  • male
  • onset age
  • overall survival
  • progression free survival
  • risk assessment
  • treatment response


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