ReviewOrphanet journal of rare diseases2013
Aggressive mature natural killer cell neoplasms: from epidemiology to diagnosis.
Review in Orphanet journal of rare diseases, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 40 papers.
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Who cites it
40 citing papers in PubMed, 91 citations in OpenAlex.
- Review
- A Masked Intruder: Extranodal Natural Killer/T-Cell Lymphoma Mimicking Preseptal Cellulitis.Mayo Clinic proceedings. Innovations, quality & outcomes · 2026Article
- Aggressive Natural Killer Cell Leukemia: A Rare and Rapidly Progressive Hematologic Malignancy-Case Report and Literature Review.Case reports in hematology · 2026Article
- Case report: a critically ill patient with aggressive NK-cell leukemia receiving emergency chemotherapy in the ICU.Virology journal · 2025Article
- Extranodal natural killer/T-cell lymphoma with tonsil involvement: a case report.BMC oral health · 2024Article
- Clinical epidemiology of Epstein-Barr virus-associated Lymphoproliferative Disorders (EBV-LPDs) in hospitalized children: A six-year multi-institutional study in China.Italian journal of pediatrics · 2024Article
- Multiple roles for AU-rich RNA binding proteins in the development of haematologic malignancies and their resistance to chemotherapy.RNA biology · 2024Review
- [Clinical characteristics and prognosis of patients with aggressive NK cell leukemia].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2022Article
- Extranodal Natural Killer/T-Cell Lymphomas: Current Approaches and Future Directions.Journal of clinical medicine · 2022Review
- Aggressive Natural Killer Cell Leukemia in an Adolescent Patient: A Case Report and Literature Review.Frontiers in pediatrics · 2022Article
- The Effect of Chronic Rhinosinusitis on the Staging and Prognosis of Extranodal Natural Killer/T-Cell Lymphoma: A Single-Center Retrospective Analysis.Frontiers in oncology · 2022Article
- Aggressive natural killer cell leukemia with skin manifestation associated with hemophagocytic lymphohistiocytosis: A case report.World journal of clinical cases · 2021Article
- NK-/T-cell lymphomas.Leukemia · 2021Review
- Extranodal NK/T-Cell Lymphoma, Nasal Type: Genetic, Biologic, and Clinical Aspects with a Central Focus on Epstein-Barr Virus Relation.Microorganisms · 2021Review
- Molecular Genetics in Epstein-Barr Virus-Associated Malignancies.Life (Basel, Switzerland) · 2021Review
- [Unilateral visual impairment with bilateral indistinct optic nerve margins].Der Ophthalmologe : Zeitschrift der Deutschen Ophthalmologischen Gesellschaft · 2021Article
- Rare Diseases of the Nose, the Paranasal Sinuses, and the Anterior Skull Base.Laryngo- rhino- otologie · 2021Review
- Extranodal NK/T cell lymphoma.Blood research · 2020Review
- NK Cell Priming From Endogenous Homeostatic Signals Is Modulated by CIS.Frontiers in immunology · 2020Article
- Activated hippo signal pathway inhibits cell proliferation and promotes apoptosis in NK/T cell lymphoma cells.Cancer medicine · 2019Article
Corrections and comments
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Authors and funding
1 author at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Mature natural killer (NK) cell neoplasms are classified by the World Health Organization into NK/T cell lymphoma, nasal type (NKTCL), aggressive NK-cell leukemia (ANKCL) and chronic lymphoproliferative disorders of NK-cells, the latter being considered provisionally. NKTCL and ANKCL are rare diseases, with higher prevalence in Asia, Central and South America. Most NKTCL present extranodal, as a destructive tumor affecting the nose and upper aerodigestive tract (nasal NKTCL) or any organ or tissue (extranasal NKTCL) whereas ANKCL manifests as a systemic disease with multiorgan involvement and naturally evolutes to death in a few weeks. The histopathological hallmark of these aggressive NK-cell tumors is a polymorphic neoplastic infiltrate with angiocentricity, angiodestruction and tissue necrosis. The tumor cells have cytoplasmatic azurophilic granules and usually show a CD45(+bright), CD2(+), sCD3(-), cytCD3epsilon(+), CD56(+bright), CD16(−/+), cytotoxic granules molecules(+) phenotype. T-cell receptor genes are in germ-line configuration. Epstein-Barr virus (EBV) -encoded membrane proteins and early region EBV RNA are usually detected on lymphoma cells, with a pattern suggestive of a latent viral infection type II. Complex chromosomal abnormalities are frequent and loss of chromosomes 6q, 11q, 13q, and 17p are recurrent aberrations. The rarity of the NK-cell tumors limits our ability to standardize the procedures for the diagnosis and clinical management and efforts should be made to encourage multi-institutional registries.
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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.