An 83-year-old man with a past medical history of initial stage chronic lymphocytic leukemia (Rai 0/Binet A) was admitted to the hospital due to nausea, vomiting, and constipation of several days’ progression. An abdominal CT scan showed thickening of the walls of the third and fourth parts of the duodenum, as well as a large aortic aneurysm extrinsically compressing the duodenum (Fig. 1). A gastroduodenoscopy revealed an almost completely ulcerated stricture in the third part of the duodenum (Figs. 2 and 3). Biopsy showed large lymphoid cells infiltrating the duodenal mucosa that were scarcely cohesive, with scant cytoplasm, positive for CD 20, and negative for CD 3 (Fig. 4), consistent with large B-cell lymphoma. Richter’s syndrome appears in 2-10% of the patients with chronic lymphocytic leukemia, and is its transformation into an aggressive lymphoma, usually diffuse large B-cell lymphoma. It generally manifests with adenopathies, splenomegaly, and worsening of B symptoms. Our patient presented with symptoms of partial obstruction and chemotherapy was begun. He received 3 cycles (2 R-miniCHOP and R-GemOx-Dex), with no improvement, and finally underwent laparoscopic gastrojejunostomy. Progression was unfavorable and in the following weeks he presented with acute abdomen secondary to extensive mesenteric ischemia. He was operated on again but died in the postoperative period.

Figure 1.

Thickening of the walls of the third and fourth parts of the duodenum.

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Figure 2.

An almost completely ulcerated stricture in the third part of the duodenum.

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Figure 3.

An almost completely ulcerated stricture in the third part of the duodenum.

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Figure 4.

Infiltration of the duodenal mucosa by large lymphoid cells.

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The gastrointestinal location of Richter’s syndrome is very rare. There are only ten cases described in the literature, at different levels of the digestive tract (stomach, colon, or rectum) and with diverse clinical manifestations, such as recurrent gastric ulcer, perforation, intestinal obstruction, and gastrointestinal bleeding.