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7 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
An aggressive T-cell lymphoma of the small bowel that arises out of coeliac disease, usually in somebody whose coeliac disease was diagnosed late or has not responded to a gluten-free diet. It often announces itself as a perforation or obstruction of the bowel in a person who is already underweight, which is why treatment has to deal with nutrition at the same time as the lymphoma.
What it is. A lymphoma of the T cells that live between the cells lining the small bowel. It arises in people with coeliac disease, the immune reaction to gluten, and particularly in those whose disease was diagnosed in adulthood or whose symptoms have not settled on a gluten-free diet. It is the reason coeliac disease that stops responding to the diet is investigated rather than managed.
How it presents, and why that shapes the treatment. WHO-HAEM5 tabulates the presentation: abdominal symptoms, with perforation or obstruction of the bowel common, deep involvement of the bowel wall, and a tumour made of pleomorphic large or medium cells against a prominent inflammatory background. A substantial proportion of patients come to attention as a surgical emergency. They are usually malnourished before the lymphoma starts, because the coeliac disease has been damaging the bowel, and chemotherapy in a malnourished person with a bowel at risk of perforating is a different proposition from chemotherapy in a well person. Nutritional support, often intravenous, and surgical involvement are part of the treatment and not an afterthought.
| Setting | Approach | Guideline |
|---|---|---|
| Diagnosis, and the coeliac disease behind it | The diagnosis is often made on bowel resected as an emergency for perforation or obstruction. Where there is time, it is made at endoscopy with biopsies of the small bowel. Coeliac disease is confirmed or newly diagnosed at the same time, and the rest of the family is offered testing. Staging uses the gastrointestinal system that counts depth and node involvement, and imaging of the whole abdomen matters because disease is often multifocal. | not mapped |
| Nutrition and the surgical risk, which come first | Most patients are malnourished before the lymphoma starts, because the coeliac disease has been damaging the bowel, and the bowel is at risk of perforating during treatment. Nutritional assessment and support, often intravenous, and early involvement of a surgeon are part of the treatment rather than an afterthought, and they determine what chemotherapy is possible. A strict gluten-free diet is continued throughout. | not mapped |
| Systemic treatment | In the population-based series from northern England and Scotland, conventional anthracycline-based chemotherapy with or without surgery gave a median progression-free survival of 3.4 months and overall survival of 7.1 months in 54 patients. From 1998 the same group gave patients fit enough for it ifosfamide, etoposide and epirubicin alternating with methotrexate, followed by an autologous stem cell transplant; in 26 patients treated that way, five-year progression-free survival was 52 per cent and overall survival 60 per cent. That is a comparison against a historical group from the same region rather than a randomised trial, and it is the best evidence this disease has. A clinical trial is a reasonable first choice. | not mapped |