ArticleBlood2014
ALK-negative anaplastic large cell lymphoma is a genetically heterogeneous disease with widely disparate clinical outcomes.
Article in Blood, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 199 papers, 2 of them syntheses that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
199 citing papers in PubMed, 2 syntheses or guidelines pooled it, 460 citations in OpenAlex.
- A genetic profiling guideline to support diagnosis and clinical management of lymphomas.Clinical & translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico · 2024Guideline
- Best Practices Guideline for the Pathologic Diagnosis of Breast Implant-Associated Anaplastic Large-Cell Lymphoma.Journal of clinical oncology : official journal of the American Society of Clinical Oncology · 2020Guideline
- 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
- Trial
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- A phase II study of cyclophosphamide, etoposide, vincristine and prednisone (CEOP) Alternating with Pralatrexate (P) as front line therapy for patients with peripheral T-cell lymphoma (PTCL): final results from the T- cell consortium trial.British journal of haematology · 2016Trial
- Phase II trial of dose-adjusted EPOCH in untreated systemic anaplastic large cell lymphoma.Haematologica · 2016Trial
- From molecular pathogenesis to novel Therapeutic approaches - a review of recent advances in the treatment of peripheral T cell Lymphomas.Biomarker research · 2026Review
- Article
- Gastrointestinal-Predominant ALK-Negative Systemic Anaplastic Large Cell Lymphoma Presenting as Fever of Unknown Origin: Case Report.The Korean journal of helicobacter and upper gastrointestinal research · 2026Article
- Oral manifestations of anaplastic large cell lymphoma: clinicopathological features and main immunophenotypic findings of 3 cases.Journal of hematopathology · 2026Article
- Patterns of care and clinical outcomes in systemic peripheral T-cell lymphoma: the LEO-MER prospective cohort study.Blood advances · 2026Observational
- A phase 1 trial of romidepsin, azacitidine, dexamethasone, and lenalidomide in relapsed or refractory T-cell lymphoma.Blood advances · 2026Article
- Primary Cutaneous Anaplastic Large Cell Lymphoma: A Review of Diagnosis and Treatment for the General Oncologist.Cancers · 2026Review
- Chimeric Antigen Receptor T‑Cell Therapy Targeting Tumor Necrosis Factor Receptor Superfamily Member 8 (CD30) for Relapsed or Refractory Anaplastic Large Cell Lymphoma: Rationale, Strategies, and Emerging Clinical Evidence.ACS pharmacology & translational science · 2026Review
- Genome-epigenome crosstalk in T-cell lymphomas: from maps to mechanisms.International journal of hematology · 2026Review
- Beyond the Surface: Deciphering the Role of Genetic Susceptibility in BIA-ALCL Pathogenesis.Biomedicines · 2026Review
- ALK-Negative Systemic Anaplastic Large Cell Lymphoma with Cutaneous Involvement Initially Mimicking Primary Cutaneous Disease: A Case Report.Clinical, cosmetic and investigational dermatology · 2026Article
- ALK-negative anaplastic large cell lymphoma in pediatric and adolescent patients: a mini-review.Frontiers in oncology · 2026Review
- The IL-1R and NFKBIZ pathway mediates immunoregulatory responses and immunotherapy efficacy in anaplastic large cell lymphoma.Leukemia · 2026Article
139 more citing papers are in PubMed but not listed here.
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Authors and funding
26 authors at 10 institutions in 3 countries.
Funding
Abstract
Anaplastic lymphoma kinase (ALK)-negative anaplastic large cell lymphoma (ALCL) is a CD30-positive T-cell non-Hodgkin lymphoma that morphologically resembles ALK-positive ALCL but lacks chromosomal rearrangements of the ALK gene. The genetic and clinical heterogeneity of ALK-negative ALCL has not been delineated. We performed immunohistochemistry and fluorescence in situ hybridization on 73 ALK-negative ALCLs and 32 ALK-positive ALCLs and evaluated the associations among pathology, genetics, and clinical outcome. Chromosomal rearrangements of DUSP22 and TP63 were identified in 30% and 8% of ALK-negative ALCLs, respectively. These rearrangements were mutually exclusive and were absent in ALK-positive ALCLs. Five-year overall survival rates were 85% for ALK-positive ALCLs, 90% for DUSP22-rearranged ALCLs, 17% for TP63-rearranged ALCLs, and 42% for cases lacking all 3 genetic markers (P < .0001). Hazard ratios for death in these 4 groups after adjusting for International Prognostic Index and age were 1.0 (reference group), 0.58, 8.63, and 4.16, respectively (P = 7.10 × 10(-5)). These results were similar when restricted to patients receiving anthracycline-based chemotherapy, as well as to patients not receiving stem cell transplantation. Thus, ALK-negative ALCL is a genetically heterogeneous disease with widely disparate outcomes following standard therapy. DUSP22 and TP63 rearrangements may serve as predictive biomarkers to help guide patient management.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.