• Plasma EBV DNA: Fragments of Epstein-Barr virus DNA in the blood that measure nasopharyngeal carcinoma: used to screen healthy people in endemic regions, to stage, to decide who needs extra treatment after radiotherapy, and to detect relapse.
• Prognostic Index for T-cell lymphoma (PIT): A four-item score that estimates the outlook in nodal T-cell lymphoma, built because the index used for B-cell lymphoma separated these patients poorly.
• B-cell, T-cell and NK-cell lymphoma: The first thing a lymphoma report says is which kind of lymphocyte the cancer came from.
• Epstein-Barr virus (EBV) in cancer: The common glandular-fever virus, carried lifelong by most adults, which in a minority of people drives nasopharyngeal cancer, some stomach cancers and several lymphomas.
• Lugano classification / Ann Arbor staging: The Lugano classification is the lymphoma staging system: stage I to IV by how many lymph node regions and organs are involved, with PET-based response criteria.
• Nodal and extranodal lymphoma: A lymphoma that starts in a lymph node is called nodal; one that starts in an organ is called extranodal.
• Endoscopy (EGD, EUS, ERCP): Looking inside a hollow organ with a camera on a flexible tube, taking biopsies and sometimes treating on the spot.
• Radiotherapy in lymphoma: involved-site fields, 24 Gy, 4 Gy and total skin electron therapy: Lymphoma is one of the most radiation-sensitive cancers there is, so the doses are low and the fields are small.
• The two lymphoma classifications of 2022 (WHO-HAEM5 and ICC): Since 2022 there have been two reference classifications of lymphoma rather than one, published within months of each other by overlapping groups of experts.
• The lymphoma regimen alphabet: R-CHOP, pola-R-CHP, DA-EPOCH-R, ABVD, BEACOPP and the rest: Lymphoma treatment is written in acronyms, one letter per drug.

Making the diagnosis, and why it is usually late: The presentation looks like sinusitis: blockage, bleeding, crusting and pain in the nose, often treated with antibiotics and steroids for months. A biopsy taken through the nose is often necrotic and non-diagnostic, so repeat biopsies are common and are not a failure. The diagnosis needs in situ hybridisation for Epstein-Barr-encoded RNA, which is positive in every tumour cell by definition, together with an NK-cell phenotype: CD56 positive, surface CD3 negative with cytoplasmic CD3-epsilon positive, and cytotoxic granule proteins present. A gut lesion with the same markers but no virus is the indolent NK-cell lymphoproliferative disorder of the gastrointestinal tract, which regresses on its own; WHO-HAEM5 says it is most important not to mistake one for the other.

Staging and the risk score: PET-CT of the whole body, magnetic resonance imaging of the face and sinuses to map local destruction, examination of the nose and throat, and a measurement of Epstein-Barr virus DNA in the blood, which tracks the disease and is an independent predictor of survival. The index in use is PINK, built from 527 patients treated without anthracyclines at 38 hospitals in 11 countries, which counts age over 60, stage III or IV, involvement of distant lymph nodes, and disease starting outside the nose: three-year overall survival was 81 per cent with none of those, 62 per cent with one and 25 per cent with two or more. Adding the viral DNA level gives a second version, PINK-E.

Biomarker results to ask for: Epstein-Barr virus in every tumour cell by EBER in situ hybridisation, which is part of the definition, An NK-cell phenotype: CD56 positive, surface CD3 negative with cytoplasmic CD3-epsilon positive, and cytotoxic granule proteins present, P-glycoprotein expression, which is why anthracycline chemotherapy does not work, Plasma Epstein-Barr virus DNA, which tracks the disease and is an independent predictor of survival, The PINK index: age over 60, stage III or IV, distant lymph node involvement and non-nasal disease, Deletion of 6q21-25, and mutations of the JAK-STAT pathway, epigenetic regulators, TP53, MGA and DDX3X.

Scans and tests linked to this cancer: FDG PET, Histopathology & immunohistochemistry.

Bring copies of scan reports, pathology and blood results, and a list of every medicine and supplement.

• Extranodal NK/T-cell lymphoma, nasal type: asparaginase, and why anthracyclines do not work: An Epstein-Barr virus-driven lymphoma that destroys the midline structures of the face, common in east Asia and Latin America and uncommon in Europe. It expresses P-glycoprotein, which pumps anthracyclines out of the cell, so CHOP is ineffective and must not be used. Every effective regimen contains asparaginase, which the tumour cannot resist because it lacks asparagine synthetase. Early-stage disease (stage I and II, confined to the upper aerodigestive tract) is treated with radiotherapy at a relatively high dose of about 50 Gy, given concurrently with or sandwiched between asparaginase-containing chemotherapy. Radiotherapy is the single most important component in early disease and delaying it worsens outcome. Advanced and relapsed disease is treated with an asparaginase-containing regimen. SMILE (dexamethasone, methotrexate, ifosfamide, L-asparaginase, etoposide) was tested in 38 patients with newly diagnosed stage IV, relapsed or refractory disease: the overall response rate after two cycles was 79 per cent, complete response 45 per cent, and one-year overall survival 55 per cent, with grade 4 neutropenia in 92 per cent and grade 3 or 4 infection in 61 per cent. Nineteen of the 28 who completed treatment went on to a stem cell transplant. Gentler asparaginase-based regimens such as P-GEMOX and DDGP are widely used in China. Pegylated asparaginase has largely replaced native L-asparaginase. Plasma Epstein-Barr virus DNA is used to monitor response. PD-1 blockade has substantial activity in relapsed disease and is used where available. (Asparaginase (pegaspargase, calaspargase pegol, Erwinia asparaginase), Methotrexate, Ifosfamide, Etoposide, Dexamethasone, Gemcitabine, Oxaliplatin, IMRT / IGRT (modern external beam), Radiotherapy in lymphoma: involved-site fields, 24 Gy, 4 Gy and total skin electron therapy, Pembrolizumab, Nivolumab, Epstein-Barr virus (EBV) in cancer, Plasma EBV DNA, Allogeneic stem cell transplantation)

From the standard of care recorded for this cancer; which apply depends on your stage and biomarkers. Ask which the team recommends and why.