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Every dated change on the records linked to Extranodal NK/T-cell lymphoma, newest first: approvals and regulatory steps on its medicines, trials that reported, guideline versions, milestones, and when this page itself was checked. Dates come from the records; none is inferred. Orientation, not medical advice.
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.
WHO-HAEM5 renamed the entity extranodal NK/T-cell lymphoma, recognising its presentation at several extranodal sites; the International Consensus Classification kept the nasal type qualifier.
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.
Built from 527 patients treated without anthracyclines at 38 hospitals in 11 countries: age over 60, stage III or IV, distant lymph node involvement and non-nasal disease gave three-year overall survival of 81, 62 and 25 per cent across the three risk groups.
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.
The SMILE regimen, built around asparaginase and deliberately free of anthracyclines, was tested in newly diagnosed stage IV, relapsed and refractory disease and established asparaginase as the backbone of treatment.