Nasal type extranodal NK/T-cell lymphoma
The disease is exceedingly rare in the United States and Europe but more common in Central / South America and Asia. Adults are most frequently affected, but cases in children have been reported.
EBV exposure is a key risk factor, and detection of the virus in lymphoma cells is required for diagnosis. Genetic aberrations such as deletion of chromosome 6q and possible abnormalities in the Janus kinase (JAK)-signal transducer and activator of transcription (STAT) pathway have been implicated.
Treatment is difficult and includes chemotherapy and/or radiation therapy.
Related topic: Non-Hodgkin lymphoma
M31.2 – Lethal midline granuloma
414166008 – Extranodal natural killer/T-cell lymphoma, nasal type
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Last Updated: 09/11/2019