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It most commonly develops in the nasal cavity and upper aerodigestive tract.",[68,74,78,82,86],{"key":69,"title":70,"html":71,"icon":72,"updatedAt":73},"","Overview","\u003Cp style=\"margin: 7px 0px; text-align: left;\">\u003Cspan style=\"font-size: 15px;\">Extranodal NK\u002FT-cell lymphoma (ENKTCL) is a rare and aggressive type of non-Hodgkin lymphoma. Most cases arise from natural killer (NK) cells, while a smaller proportion arise from cytotoxic T cells.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0px; text-align: left;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0px; text-align: left;\">\u003Cspan style=\"font-size: 15px;\">ENKTCL shows distinct geographic patterns and is more common in parts of Asia and Central and South America. It most often develops in the \u003Cstrong>nasal cavity, nasopharynx, paranasal sinuses, and other areas of the upper aerodigestive tract\u003C\u002Fstrong>.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0px; text-align: left;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0px; text-align: left;\">\u003Cspan style=\"font-size: 15px;\">Although ENKTCL is an aggressive lymphoma, advances in radiation therapy, asparaginase-based treatment, and newer therapies have improved treatment options. Disease stage, tumor burden, EBV DNA levels, and response to treatment are among the factors that can influence treatment decisions and outcomes.\u003C\u002Fspan>\u003C\u002Fp>","⚕️","2026-10-09",{"key":69,"title":75,"html":76,"icon":77,"updatedAt":73},"Causes","\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">The exact cause of ENKTCL is not fully understood, but the disease is \u003Cstrong>strongly associated with Epstein-Barr virus (EBV)\u003C\u002Fstrong>.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">EBV is found in the tumor cells of ENKTCL and is an important biological feature of the disease. However, having an EBV infection does not mean that someone will develop ENKTCL. EBV infection is very common, while ENKTCL remains rare.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp>\u003Cspan style=\"font-size: 15px;\">Genetic and molecular changes in lymphoma cells, as well as changes in the immune microenvironment, are also involved in the development of ENKTCL and continue to be studied.\u003C\u002Fspan>\u003C\u002Fp>","🧬",{"key":69,"title":79,"html":80,"icon":81,"updatedAt":73},"Signs and Symptoms","\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Symptoms of ENKTCL depend on where the lymphoma develops and how far the disease has spread.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">When ENKTCL involves the nasal cavity or upper aerodigestive tract, symptoms may include: \u003C\u002Fspan>\u003Cspan style=\"font-size: 15px;\">Persistent nasal congestion, \u003C\u002Fspan>\u003Cspan style=\"font-size: 15px;\">Nosebleeds, \u003C\u002Fspan>\u003Cspan style=\"font-size: 15px;\">Swelling in the nose or nasopharynx, \u003C\u002Fspan>\u003Cspan style=\"font-size: 15px;\">Facial swelling or pain, \u003C\u002Fspan>\u003Cspan style=\"font-size: 15px;\">Sores or ulcers in the nose, mouth, or palate that do not heal, \u003C\u002Fspan>\u003Cspan style=\"font-size: 15px;\">Throat discomfort or difficulty swallowing.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0 7px 48px;\">\u003Cspan style=\"font-size: 15px;\"> \u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Some people may also develop \u003Cstrong>B symptoms\u003C\u002Fstrong>, including: \u003C\u002Fspan>\u003Cspan style=\"font-size: 15px;\">Unexplained fever, \u003C\u002Fspan>\u003Cspan style=\"font-size: 15px;\">Drenching night sweats, \u003C\u002Fspan>\u003Cspan style=\"font-size: 15px;\">Unexplained weight loss.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;text-align: justify;\">\u003Cbr\u002F>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">ENKTCL can also develop outside the nasal area, including the skin, gastrointestinal tract, or testes. Symptoms vary depending on the part of the body involved.\u003C\u002Fspan>\u003C\u002Fp>","📋",{"key":69,"title":83,"html":84,"icon":85,"updatedAt":73},"Diagnosis","\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">If ENKTCL is suspected because of symptoms such as persistent nasal congestion, a mass in the nasal cavity or nasopharynx, or a nonhealing ulcer, a \u003Cstrong>tissue biopsy\u003C\u002Fstrong> is usually needed to confirm the diagnosis. Imaging and EBV-related testing can then help evaluate the extent of the disease.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Ch3 style=\"margin-top: 7px;margin-bottom: 7px;\">\u003Cspan style=\"font-size: 15px;\">\u003Cstrong>Biopsy\u003C\u002Fstrong>\u003C\u002Fspan>\u003C\u002Fh3>\u003Cp style=\"margin: 7px 0;\">\u003Cbr\u002F>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">A tissue biopsy is essential for diagnosing ENKTCL.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">A tissue sample from the affected area is examined by a pathologist. Immunohistochemistry and EBV-related testing are used to help identify the characteristics of the lymphoma cells.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">ENKTCL may show features such as angiocentric growth, blood vessel destruction, and tissue necrosis. Because some lesions contain extensive necrosis, an additional biopsy may occasionally be needed to obtain enough viable tissue for diagnosis.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Ch3 style=\"margin-top: 7px;margin-bottom: 7px;\">\u003Cspan style=\"font-size: 15px;\">\u003Cstrong>EBV Testing\u003C\u002Fstrong>\u003C\u002Fspan>\u003C\u002Fh3>\u003Cp style=\"margin: 7px 0;\">\u003Cbr\u002F>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Testing for EBV in tumor tissue is an important part of the pathological diagnosis of ENKTCL.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cstrong>EBV DNA levels\u003C\u002Fstrong> in peripheral blood can also be measured using quantitative PCR. EBV DNA can be assessed before treatment and monitored over time to help evaluate treatment response and changes in the disease.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Ch3 style=\"margin-top: 7px;margin-bottom: 7px;\">\u003Cspan style=\"font-size: 15px;\">\u003Cstrong>PET\u002FCT and Other Imaging Tests\u003C\u002Fstrong>\u003C\u002Fspan>\u003C\u002Fh3>\u003Cp style=\"margin: 7px 0;\">\u003Cbr\u002F>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">PET\u002FCT can help show where lymphoma is located throughout the body and is an important tool for staging ENKTCL and assessing treatment response.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">CT or MRI may also be used to evaluate disease involving the nasal cavity, sinuses, face, or nearby structures and can provide additional information for treatment planning, particularly radiation therapy.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Ch3 style=\"margin-top: 7px;margin-bottom: 7px;\">\u003Cspan style=\"font-size: 15px;\">\u003Cstrong>Blood and Other Tests\u003C\u002Fstrong>\u003C\u002Fspan>\u003C\u002Fh3>\u003Cp style=\"margin: 7px 0;\">\u003Cbr\u002F>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Blood tests can measure red blood cells, white blood cells, and platelets and assess liver and kidney function, lactate dehydrogenase (LDH), and other markers.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Additional tests may be performed depending on the location and extent of the disease.\u003C\u002Fspan>\u003C\u002Fp>","🩺",{"key":69,"title":87,"html":88,"icon":89,"updatedAt":73},"Treatment","\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Treatment for ENKTCL depends on several factors, including \u003Cstrong>disease stage, tumor location and burden, overall health, and response to treatment\u003C\u002Fstrong>.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Treatment strategies differ between localized and advanced disease. Radiation therapy and \u003Cstrong>asparaginase-based systemic therapy\u003C\u002Fstrong> are important components of ENKTCL treatment. Traditional CHOP or CHOP-like chemotherapy is generally not used as standard treatment for ENKTCL.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Ch3 style=\"margin-top: 7px;margin-bottom: 7px;\">\u003Cspan style=\"font-size: 15px;\">\u003Cstrong>Treatment for Localized Disease\u003C\u002Fstrong>\u003C\u002Fspan>\u003C\u002Fh3>\u003Cp style=\"margin: 7px 0;\">\u003Cbr\u002F>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">For localized ENKTCL, \u003Cstrong>radiation therapy is a central part of treatment\u003C\u002Fstrong>.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">For patients who are able to receive systemic therapy, radiation is often combined with an asparaginase-containing treatment regimen. Radiation and systemic therapy may be given concurrently, sequentially, or in other combinations depending on the disease and the individual patient.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Radiation therapy alone may be considered for selected patients with low-burden stage I disease or for those who are unable to tolerate systemic therapy.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Ch3 style=\"margin-top: 7px;margin-bottom: 7px;\">\u003Cspan style=\"font-size: 15px;\">\u003Cstrong>Treatment for Advanced Disease\u003C\u002Fstrong>\u003C\u002Fspan>\u003C\u002Fh3>\u003Cp style=\"margin: 7px 0;\">\u003Cbr\u002F>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">For advanced ENKTCL, treatment is generally based on \u003Cstrong>multi-drug regimens that include asparaginase\u003C\u002Fstrong>.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Different regimens containing pegaspargase or L-asparaginase may be considered depending on the characteristics of the disease and the patient’s overall health. Radiation therapy may also be used in selected cases depending on disease location and treatment response.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Newer approaches, including \u003Cstrong>PD-1\u002FPD-L1–directed immunotherapy\u003C\u002Fstrong>, are also being studied and used in ENKTCL, particularly for relapsed or refractory disease.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Ch3 style=\"margin-top: 7px;margin-bottom: 7px;\">\u003Cspan style=\"font-size: 15px;\">\u003Cstrong>Relapsed or Refractory ENKTCL\u003C\u002Fstrong>\u003C\u002Fspan>\u003C\u002Fh3>\u003Cp style=\"margin: 7px 0;\">\u003Cbr\u002F>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Some patients may experience a relapse after treatment or may not respond adequately to initial therapy.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">For relapsed or refractory ENKTCL, treatment decisions depend on factors such as previous therapies, the timing and location of relapse, EBV DNA levels, and overall health.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Treatment options may include other asparaginase-based regimens, immunotherapy, hematopoietic stem cell transplantation, or clinical trials.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Ch3 style=\"margin-top: 7px;margin-bottom: 7px;\">\u003C\u002Fh3>\u003Ch3 style=\"margin-top: 7px;margin-bottom: 7px;\">\u003Cspan style=\"font-size: 15px;\">\u003Cstrong>Hematopoietic Stem Cell Transplantation (HSCT)\u003C\u002Fstrong>\u003C\u002Fspan>\u003C\u002Fh3>\u003Cp style=\"margin: 7px 0;\">\u003Cbr\u002F>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Hematopoietic stem cell transplantation is not needed for everyone with ENKTCL and is \u003Cstrong>not routinely used as first-line treatment for localized disease\u003C\u002Fstrong>.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">For selected patients with advanced, high-risk, or relapsed\u002Frefractory ENKTCL who achieve a good response to treatment and are eligible for transplantation, HSCT may be considered.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Depending on disease status, response to previous treatment, overall health, and donor availability, either \u003Cstrong>autologous or allogeneic HSCT\u003C\u002Fstrong> may be considered. The decision about whether to proceed with transplantation and which type to use requires individualized assessment.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Ch3 style=\"margin-top: 7px;margin-bottom: 7px;\">\u003C\u002Fh3>\u003Ch3 style=\"margin-top: 7px;margin-bottom: 7px;\">\u003Cspan style=\"font-size: 15px;\">\u003Cstrong>Clinical Trials\u003C\u002Fstrong>\u003C\u002Fspan>\u003C\u002Fh3>\u003Cp style=\"margin: 7px 0;\">\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Because ENKTCL is rare, treatment continues to evolve. Clinical trials are studying new immunotherapies, targeted therapies, and treatment combinations.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">For people with advanced, relapsed, or refractory disease, discussing available clinical trials with the healthcare team may help identify additional treatment options.\u003C\u002Fspan>\u003C\u002Fp>","💊",{"author":69,"reviewedBy":69,"lastReviewed":69,"datePublished":69,"citations":91,"disclaimer":69},[],false,1792294327753]