Obscure Gastrointestinal Bleeding and Video Capsule Retention Due to Enteropathy-Associated T-Cell Lymphoma

Small bowel tumors are a rare cause of occult gastrointestinal (GI) bleeding, at times requiring hospitalization and blood transfusion. Capsule endoscopy is a commonly used tool for investigation of occult GI bleeding. The risk of capsule retention is rare and not commonly seen when capsule endoscopy is performed for the diagnostic evaluation of occult GI bleeding. We report a case of enteropathy-associated T-cell lymphoma (EATL) discovered via examination for occult GI bleeding requiring hospitalization and subsequent capsule retention that necessitated urgent surgical management secondary to bowel obstruction.

Case Report

A 77-year-old man with a medical history significant for chronic obstructive pulmonary disease, diabetes, and mechanical aortic valve replacement maintained on warfarin was admitted to the hospital with fatigue and anemia. Compared with 1 month prior, the patients hemoglobin level decreased from 9.3 g/dL to 6.8 g/dL. His international normalized ratio was 1.7. The patient denied having frank GI bleeding; however, his stools were positive for fecal occult blood. Warfarin therapy was withdrawn, and an intravenous heparin drip was started. The patient underwent upper and lower endoscopy. No bleeding lesions were observed.

A capsule endoscopy for examination of obscure GI bleeding was then performed. The capsule endoscopy revealed red clots, ulceration, and markedly abnormal tissue estimated to be localized to the midjejunum (Figure 1) at approximately 35 minutes into small bowel transit. The capsule did not pass into the cecum. The presumptive diagnosis of a bleeding jejunal tumor was made. The patient was scheduled for a double-balloon enteroscopy, and a surgical consultation was obtained.

Figure 1.

A capsule endoscopy revealed red clots, ulceration, and markedly abnormal tissue estimated to be localized to the midjejunum.

Twelve hours following the introduction of the video capsule, the patient was noted to have severe abdominal pain with a distended abdomen. An urgent computed tomography scan of the abdomen and pelvis with contrast demonstrated focal distension of a segment of proximal jejunum and pneumatosis involving a second loop (Figure 2). An emergent, exploratory laparotomy was performed, which demonstrated a contained perforation with a segment of small bowel with full thickness ischemic necrosis. No discrete mass was identified. Forty inches of small bowel was resected, and the capsule was retrieved. The patient was eventually discharged home on Postoperative Day 23.

Figure 2.

A computed tomography scan of the abdomen and pelvis demonstrated focal distension of a segment of proximal jejunum and pneumatosis involving a second loop.

On gross pathologic examination of the resected tissue, there was a 15.5-cm area of small bowel narrowing with denuded mucosa and wall thickening of 0.1 cm. Histologic examination demonstrated a dense lymphocytic infiltrate with ischemic changes extending to the serosal layer (Figure 3). The final immunologic phenotypic profile demonstrated CD3+, CD8+, CD4-, CD56+, CD7+, EBV-, CD20-, and CD30- cells, suggestive of EATL, type II.

Figure 3.

Resected jejunum showed dense lymphocytic infiltrate with ischemic changes (hematoxylin and eosin stain, 400x magnification).

Discussion

EATL is an uncommon, intestinal tumor of T lymphocytes. It comprises only 10—25% of all primary small bowel lymphomas. The most common EATL, type I, is highly associated with celiac disease and thus presents with related symptoms. Type II EATL occurs sporadically and more often presents with obstruction or perforation of the small bowel, with no known association with celiac disease.¹ EATL typically involves the small intestine, specifically the proximal jejunum, and, less commonly, intra-abdominal lymph nodes and the colon. Abdominal pain, weight loss, and fatigue are common findings at disease presentation. Patients with untreated gluten-sensitive enteropathy are at increased risk for development of EATL. Accordingly, patients with EATL rarely have a long history of celiac disease.²

The general prognosis for EATL is poor, with reported 2-year survival rates of 15—20%. The tumor has rapid growth and a tendency to metastasize.³ Complications of intestinal perforation due to refractory ulcers occur in many patients; indeed, EATL is often accompanied by mucosal ulceration as the only endoscopic manifestation of lymphoma. The malignant transformation of intraepithelial T cells into a monoclonal population of cells with an abnormal phenotype leads to the spectrum of refractory celiac disease, ulcerative jejunitis, and finally EATL. Treatment typically involves systemic chemotherapy with consideration for hematopoietic stem cell transplant. The high incidence of postsurgical complications—as most cases are diagnosed via laparotomy—often leads to progressive deterioration, particularly in the setting of malnourishment at the time of diagnosis.⁴

To our knowledge, there has only been 1 reported case of EATL type II diagnosed with capsule endoscopy. The reported patient had refractory celiac disease, for which there was underlying high suspicion; double-balloon enteroscopy was used for histologic diagnosis.⁵ There are also case reports that describe capsule endoscopic findings of EATL following chemotherapy.^6,7

Obscure GI bleeding accounts for 5% of cases of GI bleeding.⁸ In a large portion of these cases, the source is the small bowel. In this case, given the patient’s older age, typical causes such as vascular lesions or ulcers would have been suspected rather than a small bowel tumor, which is a common cause of obscure GI bleeding in younger patients.⁹ Wireless video capsule endoscopy is a common useful diagnostic tool used when standard conventional upper and lower endoscopy fail to yield a diagnosis.¹⁰ In contrast to enteroscopy, wireless video capsule endoscopy is noninvasive and permits examination of the entire small bowel, which may otherwise be technically difficult. Furthermore, capsule endoscopy has been shown to be effective in detecting small bowel tumors.¹¹

Capsule retention is a reported risk; however, this is a rare complication. In a large review, this risk appeared to be higher in patients with definite or suspected Crohn’s disease or neoplastic lesions, although the pretest suspicion for a small bowel tumor in our patient was low.¹² With the development of patency capsules and the possibility of retrieval of retained capsules with double-balloon enteroscopy, the occurrence and clinical consequences of retention are diminished. The most commonly cited incidence of capsule retention in patients with obscure GI bleeding is 0.75%, although the percentage has been reported to be up to 5.8%.¹³ Acute obstruction due to capsule retention is rarely reported in the literature.¹⁴

Conclusion

To our knowledge, this is the first report of a new EATL diagnosis via capsule endoscopy for occult GI bleeding. Furthermore, the development of acute bowel obstruction due to capsule retention at the site of a small bowel tumor is extremely rare. We believe that this case report highlights the importance of maintaining a broad differential for obscure GI bleeding and the utility of capsule endoscopy in the diagnosis of small bowel pathology. Although small bowel tumors are relatively uncommon in elderly patients, they are relatively common in the evaluation of obscure GI bleeding. While transient retention of a video capsule can be expected at a stricture or mass that ultimately requires surgical resection, this case demonstrates the possibility of true obstruction requiring urgent surgery. Consideration for small bowel imaging prior to capsule placement has been reported, although this practice is not typically recommended.¹⁵