Ileal MALT lymphoma causing massive gastrointestinal haemorrhage revealed on computed tomography angiography
- 1Department of General Surgery, Barnet General Hospital, London, UK
- 2Department of Medicine, University College London, London, UK
- 3Department of Radiology, Barnet General Hospital, London, UK
- Dr D Marks, Department of Medicine, University College London, London WC1E 6JJ, UK;
- Received 9 October 2008
- Accepted 13 December 2008
Primary MALT lymphomas affecting the ileum are rare, and their presentation with massive haemorrhage exceptional. This report describes such a case. The patient presented with melaena and haemodynamic instability, but normal upper gastrointestinal endoscopy. Subsequent imaging with multi-detector row computed tomography angiography both localised the bleeding source to the ileum and identified the underlying tumour, resulting in considerably earlier introduction of appropriate management. The patient made an excellent recovery and remains in remission.
In cases of upper gastrointestinal haemorrhage in which endoscopy is unrevealing, the optimal sequence of subsequent investigations is undecided. Selective angiography remains the gold standard, but computed tomography (CT) angiography has an increasing role.1 The advantage of the latter is that, in addition to localising haemorrhage, it can provide anatomical information about the underlying source. It is also more readily available, particularly out-of-hours in non-tertiary care centres. We describe a patient presenting with significant haemorrhage but normal endoscopy. Early use of CT angiography not only identified the site of blood loss as the mid ileum but the appearances also suggested that this originated from a tumour, which was resected and subsequently proven to be a mucosa associated lymphoid tissue (MALT) lymphoma. Ileal MALT lymphomas are uncommon, and their presentation with massive haemorrhage extremely rare.2 This case is therefore instructive in two ways: it both highlights an unusual presentation of an uncommon disease; and illustrates how first line use of CT angiography in endoscopy negative gastrointestinal bleeding accelerated diagnosis and management of an underlying tumour.
A 56-year-old woman was admitted with a history of several hours passage of large volumes of melaena. She had no past history of gastrointestinal or hepatic disease, and no known risk factors for peptic ulceration or variceal bleeding. On examination, the patient was alert and orientated, and tachycardic though maintaining a normal blood pressure. Clinically there was significant pallor and her haemoglobin on admission was 9.0 g/dl (5.6 mmol/l). This dropped over the next 5 h to 6.9 g/dl (4.3 mmol/l) and she was subsequently transfused with 4 units of blood. She underwent an urgent oesophagogastroduodenoscopy, which did not reveal the source of the haemorrhage. Selective angiography was not immediately available as the patient presented out-of-hours, and consequently CT angiography was performed. This demonstrated contrast extravasation into the lumen of the ileum, in association with localised small bowel thickening and a 10 mm adjacent mesenteric lymph node (fig 1), suggestive of a tumour.
Exploratory laparotomy was therefore conducted, at which a mid ileal tumour 60 cm proximal to the ileocaecal valve was resected and an ileo–ileal anastomosis fashioned. Histological examination showed diffuse infiltration by centrocyte-like cells throughout the lamina propria into the entire intestinal wall, and extending to include mesenteric fat (fig 2A,B). Local lymph nodes were also involved. Immunohistochemistry further characterised the tumour as a low grade B cell MALT lymphoma (CD20+ IgM+ IgD− CD79a+ BCL2+; fig 2C,D). The proliferation index was <10%. Resection margins were clear, and staging investigations (including CT imaging of the thorax, abdomen and pelvis, and bone marrow sampling) did not demonstrate any further macroscopic tumour extension or unequivocal distant metastases.
The patient made an excellent postoperative recovery and, given the nodal involvement, subsequently received six cycles of adjuvant chemotherapy with rituximab, cyclophosphamide, vincristine and prednisolone. Her Helicobacter pylori status is negative, and the patient remains in remission.
The gastrointestinal tract is the most common extranodal site for non-Hodgkin lymphomas, which predominantly affect the stomach. Primary lymphoma of the ileum is rare in adults in western countries,2 and most case series of small intestinal lymphoma are limited in their size. The main presentations are either minor haematochezia or incidental discovery in asymptomatic patients under investigation for other reasons.3 Diarrhoea and bowel obstruction are other reported manifestations.4 Massive haemorrhage, as seen in this patient, is exceptional.
Endoscopically, most MALT lymphomas appear as sessile, semi-pedunculated protrusions.3 These are typically covered by macroscopically normal mucosa, although overlying vascular ectasia or ulceration has been reported; lesions may be multiple. Histological features include expansion of a centrocyte-like population of lymphocytes within follicles, with epithelial infiltration.5 The majority are B cell derived, and are positive for CD20, CD79a and immunoglobulins.
The pathogenesis of sporadic intestinal MALT lymphomas is incompletely understood. Within the stomach, they are associated with H pylori infection, eradication of which often leads to regression of low grade lesions.6 This relationship is weaker in other regions of the bowel, where antibiotic therapy is less effective. Other risk factors for small bowel lymphomas include malabsorption syndromes, inflammatory bowel disease and immunodeficiency, none of which were present in our patient. MALT lymphomas are typically relatively indolent and the prognosis good, with 5 year survival >85% and progression-free survival >50%.7
Multi-detector row CT angiography is a minimally invasive radiological technique capable of high definition imaging of the mesenteric vasculature following administration of intravenous contrast. Faster acquisition times and thin slice collimation protocols associated with powerful processing software result in greatly improved spatial resolution and image quality over conventional CT scanning.8 This case is particularly instructive as it illustrates how this modality was able not only to locate the source of a large upper gastrointestinal bleed undetectable at endoscopy, but also indicate the underlying diagnosis. This would not have been possible with the alternative techniques of selective angiography or red cell scintigraphy. Video capsule endoscopy may be capable of providing some similar anatomical information, but is not appropriate for emergency use. CT angiography is perhaps of even greater relevance in district general hospital settings, where access to these other investigations is often limited, especially out-of-hours.1 Its first line use in our patient was also noteworthy as the diagnosis was unsuspected, since MALT lymphomas rarely present with profuse haemorrhage.2 Early recognition of the tumour considerably accelerated institution of appropriate immediate and long term management.
Key learning points
Mucosa associated lymphoid tissue lymphomas can present with massive gastrointestinal bleeding.
In endoscopy negative upper gastrointestinal haemorrhage, CT angiography may supersede invasive selective angiography as the first line investigation.
CT angiography may reveal the anatomic source of haemorrhage as well as its location, which can accelerate diagnosis and definitive treatment.
This report therefore adds to the body of evidence advocating use of CT angiography as the investigation of choice in patients with upper gastrointestinal bleeds but non-diagnostic endoscopic studies. In emergency scenarios, its sensitivity and specificity can be as high as 91% and 99% respectively,9 10 in theory capable of detecting bleeds with flow rates as low as 0.2 ml/min.11 These can be higher than those of oesophagogastroduodenoscopy in cases of profuse haemorrhage in which bleeding rates exceed 1 ml/min, when the precise focus can be difficult to visualise; one study reported that diagnosis could not be achieved in up to 24% of patients at first endoscopy.12 The drawback of CT angiography is that it cannot be used therapeutically to terminate the haemorrhage, for example, by embolisation, which can be achieved with conventional selective catheter angiography. It can, however, provide a useful tool to guide subsequent definitive treatment.
Theoretically, there may be an increased risk of adverse reaction to the repeat administration of contrast medium required if undertaking CT angiography that might subsequently lead to a therapeutic interventional angiographic procedure. Nephrotoxicity is a particular concern in patients with hypovolaemic pre-renal failure secondary to haemorrhage, and appropriate fluid resuscitation is mandatory. Nonetheless, fewer runs with relatively small doses of contrast are needed for the therapeutic selective angiography when guided by previous CT, so the total amount required is usually similar to that of a longer diagnostic and therapeutic conventional angiogram.13
This case both highlights and advocates the use of CT angiography as a first line investigation for upper gastrointestinal bleeds in situations where endoscopy is unrewarding. It also serves as a reminder that MALT lymphomas can rarely present with catastrophic haemorrhage.