ArticleHaematologica2023
ALK-negative anaplastic large cell lymphoma with
Article in Haematologica, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 26 citations in OpenAlex.
- Brentuximab vedotin plus chemotherapy for the treatment of front-line systemic anaplastic large cell lymphoma: subgroup analysis of the ECHELON-2 study at 5 years' follow-up.Blood cancer journal · 2025Trial
- Article
- ALK-negative anaplastic large cell lymphoma in pediatric and adolescent patients: a mini-review.Frontiers in oncology · 2026Review
- Personalized Immunotherapy for T Cell Lymphomas: From Immune Escape to Precision Therapeutics.Journal of personalized medicine · 2025Review
- Epidemiology of mature T-cell and NK-cell neoplasms: east and west.The Lancet regional health. Western Pacific · 2025Review
- Establishment and characterization of a novel patient-derived TP63-rearranged anaplastic large-cell lymphoma model PTCL-S1.Human cell · 2025Article
- Distribution reappraisal of peripheral T- and NK-cell lymphoma entities through the French Lymphopath network database.HemaSphere · 2025Article
- Molecular Insights into the Diagnosis of Anaplastic Large Cell Lymphoma: Beyond Morphology and Immunophenotype.International journal of molecular sciences · 2025Review
- Review
- Clinicopathologic features of primary central nervous system anaplastic large cell lymphoma: a multicenter study identifies age and ALK status as prognostic factors.Journal of hematopathology · 2024Article
- Review
- A practical approach to the modern diagnosis and classification of T- and NK-cell lymphomas.Blood · 2024Review
- Small cell pattern of ALK-negative anaplastic large cell lymphoma with double-hit rearrangements ofEJHaem · 2024Article
- Diverse and reprogrammable mechanisms of malignant cell transformation in lymphocytes: pathogenetic insights and translational implications.Frontiers in oncology · 2024Review
- What is new in the classification of peripheral T cell lymphomas?Pathologie (Heidelberg, Germany) · 2023Review
- Pathobiology of nodal peripheral T-cell lymphomas: current understanding and future directions.Haematologica · 2023Review
- Introduction to the peripheral T-cell lymphoma review series: advances in molecular characterization, classification refinement and treatment optimization.Haematologica · 2023Article
- Anaplastic large cell lymphomas with equivocal DUSP22 FISH results: recommendations for clinical reporting and diagnostic evaluation.Human pathology · 2023Article
- Article
- [Classification of peripheral T-cell lymphomas : News and open questions].Pathologie (Heidelberg, Germany) · 2023Review
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Authors and funding
23 authors at 11 institutions in 4 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
ALK-negative anaplastic large cell lymphoma (ALCL) comprises subgroups harboring rearrangements of DUSP22 (DUSP22- R) or TP63 (TP63-R). Two studies reported 90% and 40% 5-year overall survival (OS) rates in 21 and 12 DUSP22-R/TP63- not rearranged (NR) patients, respectively, making the prognostic impact of DUSP22-R unclear. Here, 104 newly diagnosed ALK-negative ALCL patients (including 37 from first-line clinical trials) from the LYSA TENOMIC database were analyzed by break-apart fluorescence in situ hybridization assays for DUSP22-R and TP63-R. There were 47/104 (45%) DUSP22-R and 2/93 (2%) TP63-R cases, including one DUSP22-R/TP63-R case. DUSP22-R tumors more frequently showed CD3 expression (62% vs. 35%, P=0.01), and less commonly a cytotoxic phenotype (27% vs. 82%; P<0.001). At diagnosis, DUSP22- R ALCL patients more frequently had bone involvement (32% vs. 13%, P=0.03). The patient with DUSP22-R/TP63-R ALCL had a rapidly fatal outcome. After a median follow-up of 4.9 years, 5-year progression-free survival (PFS) and OS rates of 84 patients without TP63-R treated with curative-intent anthracycline-based chemotherapy were 41% and 53%, respectively. According to DUSP22 status, 5-year PFS was 57% for 39 DUSP22-R versus 26% for 45 triple-negative (DUSP22-NR/TP63-NR/ALK-negative) patients (P=0.001). The corresponding 5-year OS rates were 65% and 41%, respectively (P=0.07). In multivariate analysis, performance status and DUSP22 status significantly affected PFS, and distinguished four risk groups, with 4-year PFS and OS ranging from 17% to 73% and 21% to 77%, respectively. Performance status but not DUSP22 status influenced OS. The use of brentuximab vedotin in relapsed/refractory patients improved OS independently of DUSP22 status. Our findings support the biological and clinical distinctiveness of DUSP22- R ALK-negative ALCL. Its relevance to outcome in patients receiving frontline brentuximab vedotin remains to be determined.
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