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Description
A 74-year-old woman has visited to our hospital with general lassitude and appetite loss in October 2010. The patient had experienced abdominal pain for the past 2 months and had become aware of insidious weight loss of 5 kg in the last few months. The abdomen was flat and soft, and bowel sounds were slightly hyperactive. There was no history of serious illnesses, operations or hospitalisations. Blood analyses showed a haemoglobin level of 9.2 g/dl, and biochemical analysis showed a serum lactate dehydrogenase level of 201 IU/l and an albumin level of 2.5 g/dl. CT revealed thickening of the wall in the small intestine without intestinal occlusion and lymphadenopathy of the mesenteric lymph nodes (figure 1). The patient then underwent the lower double balloon enteroscopy, and revealed stenosis of the small intestine by using gastrografin, though the lesion could not reached. The patient underwent partial resection of the small intestine and lymph node resection of the mesenteric lymph nodes. The pathological specimen showed diffuse mucosal infiltration by slightly large neoplastic cells with round nuclei having a stippled chromatin pattern and villous atrophy around the anal side of the resected small intestine. The neoplastic cells expressed CD3, TIA-1 and granzyme B (figure 2). These results suggested enteropathy-associated T cell lymphoma. Findings of the mesenteric lymph nodes were negative for lymphoma cells. The patient was staged as IB, and treated with six cycles of a regimen comprising pirarubicin-cyclophosphamide-vincristine-predonisolone therapy, and remained in remission until the last follow-up evaluation in November 2011.
CT of the abdomen revealed thickening of the wall in the small intestine and lymphadenopathy of the mesenteric lymph nodes.
Enteropathy-associated T cell lymphoma histology in this case. (A) Gross inspection of the surgical specimen revealed a stenotic segment in the small intestine. (B) The pathological specimen showed multiple ulcers (B, H&E stain, 20×) with diffuse mucosal infiltration (C, H&E stain, 40×) by slightly large neoplastic cells with round nuclei having a stippled chromatin pattern and villous atrophy (D, H&E stain, 100×). The neoplastic cells expressed CD3 (E, 40×).
Learning points
▶ EATL is an extremely rare T cell lymphoma that accounts for less than 1% of all non-Hodgkin's lymphomas and has a poor prognosis.1 2
▶ EATL is often diagnosed in an advanced stage, with frequent multifocal involvement of the small intestine. Delayed diagnosis leads to poor general conditions and recurrence of complications such as infections, perforations, gastrointestinal hemorrhages, and occlusions. This case was fortunately diagnosed in an early stage of the disease by CT findings in particular. A high index of suspicion is essential for early and correct diagnosis.
▶ Moreover, the most classic form of EATL is associated with celiac disease. In this case, there was a history of malabsorption lasting about 4 months, clinically similar in manifestation to celiac disease. However, these symptoms were thought to be due to lymphoma because of the disappearance of clinical signs after operation.
Footnotes
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Competing interests None.
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Patient consent Obtained.