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  Table of Contents  
Year : 2011  |  Volume : 48  |  Issue : 1  |  Page : 124-125

Enteropathy-associated T-cell lymphoma in a patient without a prior diagnosis of celiac disease: A diagnostic conundrum

1 Department of Pathology, Christian Medical College & Hospital, Ludhiana, India
2 Department of Pathology, Lousiana State University Health Sciences Center, Shreveport, LA, USA
3 Department of Surgery, Christian Medical College & Hospital, Ludhiana, India

Date of Web Publication10-Feb-2011

Correspondence Address:
S Jacob
Department of Pathology, Christian Medical College & Hospital, Ludhiana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-509X.76640

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How to cite this article:
Jacob S, Mohapatra D, Nordberg M L, Cotelingam J D, Upadhyay S, Japa D. Enteropathy-associated T-cell lymphoma in a patient without a prior diagnosis of celiac disease: A diagnostic conundrum. Indian J Cancer 2011;48:124-5

How to cite this URL:
Jacob S, Mohapatra D, Nordberg M L, Cotelingam J D, Upadhyay S, Japa D. Enteropathy-associated T-cell lymphoma in a patient without a prior diagnosis of celiac disease: A diagnostic conundrum. Indian J Cancer [serial online] 2011 [cited 2021 May 14];48:124-5. Available from: https://www.indianjcancer.com/text.asp?2011/48/1/124/76640


A 45-year old female presented with acute onset of fever, abdominal pain, and abdominal distention. There was history of chronic diarrhea and weight loss of 1 year duration, but she had not sought any prior medical attention. She was malnourished and had signs of peritonitis. She was taken up for emergency surgery; no radiological imaging was done. Laparotomy showed multiple ulcers and perforations in the jejunum and proximal ileum and multiple enlarged mesenteric lymph nodes. The involved parts of the bowel were resected and end to end anastomosis done. The clinical diagnosis was enteric fever with intestinal perforation. The patient died a day after surgery due to uncontrolled sepsis.

The resected jejunal and ileal segments showed multiple irregular, variable-sized ulcers , the largest measuring 3.5 cm × 2 cm. Some ulcers were deep perforating. Microscopic examination of the ulcers showed florid mixed inflammatory infiltrate. However, careful scrutiny revealed varying numbers of neoplastic lymphoid cells amidst the inflammatory infiltrate. The cells were mostly medium to large immunoblast-like cells with vesicular nuclei and prominent nucleoli with few small lymphocyte-like and occasional large, pleomorphic bi- and multi-nucleated cells [Figure 1]. The adjacent non-ulcerated mucosa showed scattered similar lymphoid cells. The neoplastic cells were CD3, CD7, and CD45 RO positive and negative for CD56 and CD20 [Figure 2]a−c. The large tumor cells expressed LCA, EMA, and BOB-1 and were immunonegative for CD15, CD30, pankeratin, and ALK-1. Molecular genetic studies by FISH technique were negative for ALK-1 gene rearrangement. PCR study showed clonal population T-cell receptor (gamma) gene rearrangement [Figure 2]d. The uninvolved mucosa showed subtotal villous atrophy and crypt hyperplasia, i.e., features compatible with celiac disease [[Figure 1], inset]. A diagnosis of enteropathy-associated T-cell lymphoma (EATL) was made.
Figure 1: EATL showing mixture of large immunoblast like cells, small lymphoid cells, and occasional pleomorphic bi-nucleated cells (H and E ×400). Inset: grossly uninvolved jejunal mucosa showing subtotal villous atrophy and crypt hyperplasia (H and E × 100).

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Figure 2: (a−c) Neoplastic cells showing immuno-positivity for CD3, CD45 RO, and CD7 (original magnification ×400). (d) Multiplex PCR for T-cell clonality shows abnormal clonal TCR (gamma) gene rearrangement.

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Enteropathy-associated T cell lymphoma is a rare intestinal lymphoma, which may be associated with celiac disease. [1] Refractory celiac disease may be subdivided into types I and II with a phenotypically normal and aberrant intraepithelial T-cell population, respectively. Transition of type II into EATL is common. The common presenting symptoms of EATL are abdominal pain, weight loss, and diarrhea. The interval between the onset of celiac disease and the development of EATL varies from 3 months to more than 40 years. EATL usually presents in the fifth and sixth decades. [2] The lesions mostly occur in jejunum but can involve other parts of small intestine and rarely the stomach and colon. They present as single or multiple ulcers, plaques, or strictures. Large polypoidal masses as seen in B-cell lymphoma is unusual. The neoplastic lymphoid cells may be medium to large immunoblast-like, bizarre pleomorphic multinucleated, or small lymphocyte-like. [3] The neoplastic lymphoid cells express CD3, CD7, CD45 RO, and CD103 positivity but none express CD20. There are two histologic groups of EATL, viz., (i) pleomorphic-anaplastic EATL which is often CD56 negative and is usually associated with a history of celiac disease and histologic evidence of enteropathy, (ii) monomorphic EATL which is usually CD56 positive, often occurs without a history of celiac disease, and has variable evidence of enteropathy.[4] EATL needs to be differentiated from Hodgkin's disease, due to its cellular polymorphous population and Reed−Sternberg-like cells. The associated florid inflammatory cell infiltrate can mask the neoplastic cells leading to a mis-diagnosis of inflammatory pathology. [3]

The clinical course of EATL is extremely unfavorable due to complications of multiple segmental involvement, perforation and dissemination. [2] The common sites of dissemination are liver, spleen and bone marrow. [4]

To conclude, the diagnostic dilemma of EATL is compounded by the gross presentation of only ulcerative lesions without any tumor mass and microscopically by the frequently associated exuberant inflammatory response, which masks and dilutes the diagnostic tumor cell population. Absence of a prior history of celiac disease further adds to the problem. Another major difficulty involving EATL is its small bowel location, which cannot be reached with conventional endoscopy. Small bowel imaging techniques like video capsule endoscopy or double balloon enteroscopy may be helpful screening modalities [5]

In India, 90%−95% of celiac disease (CD) remains undetected. Thus, there is a need to start studies to estimate prevalence of CD all over India. [5]

  References Top

1.Honemann D, Prince HM, Hicks RJ, Seymour JF. Enteropathy-associated T-cell lymphoma without a prior diagnosis of coeliac disease: diagnostic dilemmas and management options. Ann Hematol 2005;84:118-21.   Back to cited text no. 1
2.Al-toma A, Verbeek WHM, Hadithi M,von Blomberg BME,Mulder CJJ. Survival in refractory coeliac disease and enteropathy-associated T-cell lymphoma:retrospective evaluation of single - centre experience. Gut 2007;56:1373-8.  Back to cited text no. 2
3.Isaacson PG. Gastrointestinal lymphoma. Hum Pathol 1994;25:1020-9.  Back to cited text no. 3
4.Kataoka I, Arima F, Nishimoto J, Watanabe T, Kobayashi Y, Tamura R, Yamamoto S, et al. Enteropathy-type T-cell lymphoma showing repeated small bowel rupture and refractoriness to chemotherapy: a case report. Jpn J Clin Oncol 2002;32:546-9.  Back to cited text no. 4
5.Gupta R, Reddy DN, Makharia GK, Sood A, Ramakrishna BS, Yachha SK, et al. Indian task force for celiac disease: current status. World J Gastroenterol 2009;15:6028-33.  Back to cited text no. 5


  [Figure 1], [Figure 2]


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