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Disease Profile

Extranodal nasal NK/T cell lymphoma

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

NK/T-cell lymphoma; Angiocentric T-cell lymphoma; Lethal midline granuloma;

Categories

Blood Diseases; Skin Diseases

Summary

Extranodal nasal NK/T cell lymphoma (NKTCL) is a rare type of cancer. The term extranodal is used because this form of lymphoma is found outside of the traditional lymph node groupings. It mainly affects men around 50 years of age, and usually arises in the nose, paranasal sinuses (paranasal sinuses are cavities (spaces) or small tunnels. located around or near the nose), orbits or upper airway, and that can present with a nasal mass, nasal bleeding, nasal obstruction, a hole in the palate, and mid-facial and/or upper airway destructive lesions. In advanced disease stages, which are associated with a poor prognosis, NKTCL may affect other organs, but usually without enlarged nodes in the body.[1][2][3] The treatment depends on the extent of disease and usually involves radiotherapy and chemotherapy.[4]

Learn more

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.

References

  1. Lima M. Aggressive mature natural killer cell neoplasms: from epidemiology to diagnosis:. Orphanet Journal of Rare Diseases. 2013; 8:95. https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-8-95.
  2. Extranodal nasal NK/T cell lymphoma. Orphanet. 2017; https://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=11769.
  3. Liess BD. NK-Cell Lymphomas of the Head and Neck. Medscape Reference. 2016; https://emedicine.medscape.com/article/871609-overview.
  4. Tobinai K & Yamaguchi M. Treatment of extranodal NK/T cell lymphoma, nasal type. UpToDate. November 24, 2015; https://www.uptodate.com/contents/treatment-of-extranodal-nk-t-cell-lymphoma-nasal-type.