ArticleBlood2012
Molecular features of hepatosplenic T-cell lymphoma unravels potential novel therapeutic targets.
Article in Blood, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 50 papers.
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Who cites it
50 citing papers in PubMed, 106 citations in OpenAlex.
- Benchmarking spleen tyrosine kinase (Syk) expression in T- and NK-cell neoplasms: Implications for therapeutic targeting.British journal of haematology · 2025Article
- Hepatosplenic T-cell lymphoma in children and adolescents.Blood advances · 2025Review
- Clinical and splenectomy-based treatment outcomes in 40 cases of hepatosplenic T-cell lymphoma: a comprehensive analysis.World journal of surgical oncology · 2024Article
- Lack of SMARCB1 expression characterizes a subset of human and murine peripheral T-cell lymphomas.Nature communications · 2024Article
- Biology and genetics of extranodal mature T-cell and NKcell lymphomas and lymphoproliferative disorders.Haematologica · 2023Review
- Few and far between: clinical management of rare extranodal subtypes of mature T-cell and NK-cell lymphomas.Haematologica · 2023Review
- SOHO State of the Art Updates and Next Questions | Challenging Cases in Rare T-Cell Lymphomas.Clinical lymphoma, myeloma & leukemia · 2023Review
- Single cell profiling of γδ hepatosplenic T-cell lymphoma unravels tumor cell heterogeneity associated with disease progression.Cellular oncology (Dordrecht, Netherlands) · 2023Article
- Extranodal T- and NK-cell lymphomas.Virchows Archiv : an international journal of pathology · 2023Review
- Monomorphic epitheliotropic intestinal T-cell lymphoma comprises morphologic and genomic heterogeneity impacting outcome.Haematologica · 2023Article
- Role of cytokine in malignant T-cell metabolism and subsequent alternation in T-cell tumor microenvironment.Frontiers in oncology · 2023Review
- The role of Vav3 expression for inflammation and cell death during experimental myocardial infarction.Clinics (Sao Paulo, Brazil) · 2023Article
- How molecular advances may improve the diagnosis and management of PTCL patients.Frontiers in oncology · 2023Review
- Genetic profiling and biomarkers in peripheral T-cell lymphomas: current role in the diagnostic work-up.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2022Review
- Classification and challenges in the histopathological diagnosis of peripheral T-cell lymphomas, emphasis on the WHO-HAEM5 updates.Frontiers in oncology · 2022Review
- Review
- Chronic T cell receptor stimulation unmasks NK receptor signaling in peripheral T cell lymphomas via epigenetic reprogramming.The Journal of clinical investigation · 2021Article
- Molecularly targeted therapies for relapsed and refractory peripheral T-cell lymphomas.Seminars in hematology · 2021Article
- Review
- A case of hepatosplenic T-cell lymphoma successfully treated by HLA haploidentical stem cell transplantation.Journal of clinical and experimental hematopathology : JCEH · 2020Article
Corrections and comments
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Authors and funding
13 authors at 11 institutions in 4 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The pathogenesis of hepatosplenic T-cell lymphoma (HSTL), a rare entity mostly derived from γδ T cells and usually with a fatal outcome, remains largely unknown. In this study, HSTL samples (7γδ and 2αβ) and the DERL2 HSTL cell line were subjected to combined gene-expression profiling and array-based comparative genomic hybridization. Compared with other T-cell lymphomas, HSTL had a distinct molecular signature irrespective of TCR cell lineage. Compared with peripheral T-cell lymphoma, not otherwise specified and normal γδ T cells, HSTL overexpressed genes encoding NK-cell-associated molecules, oncogenes (FOS and VAV3), the sphingosine-1-phosphatase receptor 5 involved in cell trafficking, and the tyrosine kinase SYK, whereas the tumor-suppressor gene AIM1 (absent in melanoma 1) was among the most down-expressed. We found highly methylated CpG islands of AIM1 in DERL2 cells, and decitabine treatment induced a significant increase in AIM1 transcripts. Syk was present in HSTL cells and DERL2 cells contained phosphorylated Syk and were sensitive to a Syk inhibitor in vitro. Genomic profiles confirmed recurrent isochromosome 7q (n = 6/9) without alterations at the SYK and AIM1 loci. Our results identify a distinct molecular signature for HSTL and highlight oncogenic pathways that offer rationale for exploring new therapeutic options such as Syk inhibitors and demethylating agents.
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