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A 75-year-old woman with multifocal jejunal strictures
  1. Hui Yann Lee1,
  2. Gina Howarth2,
  3. Robert Willert1
  1. 1Department of Endoscopy, Manchester Royal Infirmary, Central Manchester University Hospitals Foundation Trust, Manchester, UK
  2. 2Department of Histopathology, Manchester Royal Infirmary, Central Manchester University Hospitals Foundation Trust, Manchester, UK
  1. Correspondence to Dr Hui Yann Lee, Department of Endoscopy, Manchester Royal Infirmary, Central Manchester University Hospitals Foundation Trust, Oxford Road, Manchester M13 9WL, UK; huiyann.lee{at}cmft.nhs.uk

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Clinical presentation

A previously fit and well 75-year-old Caucasian woman presented with a 2-month history of postprandial abdominal pain, vomiting and 10 kg weight loss without diarrhoea.

Blood investigations showed marked hypoalbuminaemia of 18 g/L and elevated C reactive protein (CRP) of 185 mg/L. Tissue transglutaminase IgA level was within normal limits without evidence of IgA deficiency. Faecal calprotectin was elevated at 205 µg/g.

Abdominal CT revealed subacute small bowel obstruction characterised by multiple long, thick walled, dilated loops of jejunum (figure 1) associated with lymphadenopathy. Histological findings from laparoscopic mesenteric lymph node resection showed reactive features only.

Figure 1

Coronal CT view of long thick-walled jejunal loops with dilatation.

Intravenous hydrocortisone was started with subsequent symptomatic improvement in tolerated oral intake, paralleled by a reduction in CRP to 25 mg/L.

Antegrade double balloon enteroscopy (DBE) was performed to directly visualise the small bowel mucosa (figure 2).

Figure 2

Endoscopic findings on antegrade double balloon enteroscopy. (A and B) Small bowel mucosa. (C) First jejunal stricture. (D) Second jejunal stricture.

Question

What is the diagnosis?

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Answer

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Antegrade DBE showed diffuse small bowel mucosal atrophy and scalloping on air insufflation (figure 2A) and on underwater immersion technique (figure 2B). Two partially obstructing ulcerative jejunal strictures were visualised each 10 cm in length (figure 2C, D).

Histology confirmed our diagnosis of enteropathy associated T cell lymphoma (EATL) (figure 3) complicating seronegative coeliac disease (figure 4). EATL is a rare non-Hodgkin’s lymphoma of the small intestine, often but not always associated with coeliac disease. EATL comprised 5.4% of lymphomas in a multicentre international study, associated with poor overall median survival of 10 months.1

Figure 3

Histological findings of ulcerated strictures—Enteropathy associated T cell lymphoma. (A) H&E stain: Diffuse infiltrate of medium-large neoplastic lymphoid cells admixed with inflammatory cells. (B) CD3 immunostain: Neoplastic cells are positive for CD3 (T cell marker). (C) Ki67 immunostain: 60%–70% neoplastic cells positive for Ki67 (high grade tumour).

Figure 4

Histological findings of jejunal mucosa—Coeliac disease. (A) Shortened villi indicating partial villous atrophy. (B) Increased intraepithelial lymphocytes.

Initial features of this case that mimicked Crohn's disease included: multifocal deep small bowel strictures (beyond the reach of conventional oesophagogastroduodenoscopy), elevated faecal calprotectin and symptomatic improvement following corticosteroids.

DBE is, therefore, a key investigation for definitive diagnosis of deep small bowel ulcerative lesions by achieving direct visualisation and tissue sampling. Mucosal biopsies permit differentiation of EATL from ulcerative jejunitis in coeliac disease.2 EATL can be difficult to diagnose in mucosal biopsies as the neoplastic cells can be masked by abundant associated inflammatory cells. Obtaining biopsies of the suspicious ulcerated areas and adjacent non-ulcerated mucosa improves the likelihood of diagnosing EATL.

References

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Footnotes

  • Contributors HYL performed the endoscopy, wrote the manuscript and prepared the endoscopic figure. GH prepared the histology figure with legends, reported the original histology slides and provided histopathology opinion for manuscript writing. RW proofread the manuscript and supervised the case.

  • Competing interests None.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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