[HTML][HTML] ALK-positive anaplastic large-cell lymphoma in adults: an individual patient data pooled analysis of 263 patients

D Sibon, DP Nguyen, N Schmitz, R Suzuki… - …, 2019 - ncbi.nlm.nih.gov
D Sibon, DP Nguyen, N Schmitz, R Suzuki, AL Feldman, R Gressin, L Lamant…
Haematologica, 2019ncbi.nlm.nih.gov
Anaplastic lymphoma kinase (ALK)-positive anaplastic large-cell lymphoma (ALCL)
comprises 0.5% of adult lymphomas. 1 In many prior retrospective adult series, despite clear
differences in outcome, ALK-positive ALCL has been pooled with ALK-negative ALCL,
impairing the exploration of prognostic factors and impact of different treatments for ALK-
positive ALCL. 2–8 Cyclophosphamide, doxorubicin, vincristine, and prednisone
(CHOP)/CHOP-like regimen remains the standard treatment of ALK-positive ALCL, with a 5 …
Anaplastic lymphoma kinase (ALK)-positive anaplastic large-cell lymphoma (ALCL) comprises 0.5% of adult lymphomas. 1 In many prior retrospective adult series, despite clear differences in outcome, ALK-positive ALCL has been pooled with ALK-negative ALCL, impairing the exploration of prognostic factors and impact of different treatments for ALK-positive ALCL. 2–8 Cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP)/CHOP-like regimen remains the standard treatment of ALK-positive ALCL, with a 5-year overall survival (OS) rate of 70-90%. 9 Most patients are young (median age 35 years) with some studies suggesting this is the predominant reason outcomes are generally more favorable than in ALK-negative ALCL. 3, 6 A retrospective study from the German High-Grade Non-Hodgkin Lymphoma Study Group (DSHNHL) and a population-based study from the Danish and Swedish lymphoma registries suggested a possible benefit from the integration of etoposide in the front-line therapy, but definitive conclusions are lacking, and the role of etoposide is still a matter of debate. 5, 10 Herein, we conducted a pooled analysis of individual patient data (IPD) from six studies of first-line therapy in adult ALK-positive ALCL to identify the key prognostic factors and treatment impact. IPD were collected and pooled from six previously published studies: two from the Lymphoma Study Association (LYSA), 4, 6 one from the DSHNHL, 5 one from Japan, 2 one from the International Peripheral T-Cell Lymphoma Project (IPTCLP), 3 and one from the Mayo Clinic. 7 Patients recruited to the six studies met criteria for diagnosis of ALK-positive ALCL by the REAL (The Revised European American Lymphoma Classification)/World Health Organization classification11, 12 with a centralized pathologic review. Eligibility criteria and statistical analyses are available in the Online
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