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Year : 2014  |  Volume : 57  |  Issue : 4  |  Page : 658-659
An unusual case of enteropathy-associated T-cell lymphoma with CD20 positivity

Department of Histopathology, BLK Hospital, New Delhi, India

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Date of Web Publication11-Oct-2014

How to cite this article:
Misra DS, Bhardwaj M, Bahuguna G, Malhotra V. An unusual case of enteropathy-associated T-cell lymphoma with CD20 positivity. Indian J Pathol Microbiol 2014;57:658-9

How to cite this URL:
Misra DS, Bhardwaj M, Bahuguna G, Malhotra V. An unusual case of enteropathy-associated T-cell lymphoma with CD20 positivity. Indian J Pathol Microbiol [serial online] 2014 [cited 2023 Nov 30];57:658-9. Available from:


We report a case of CD20-positive enteropathy-type T-cell lymphoma of a small intestine. This case was first diagnosed as celiac disease and then misdiagnosed as a B-cell lymphoma. However, molecular testing resulted in making the correct diagnosis.

A 55-year-old male patient presented with the history of malabsorption and 15 kg weight loss at another hospital. Due to blunting of villi in duodenum and dense lymphocytic infiltrate, a diagnosis of celiac disease was made at an outside hospital. He did not respond to gluten free diet. Another duodenal biopsy after 6 months at our hospital showed following pathological features.

Histological sections showed blunting of duodenal villi [Figure 1]. Dense lymphocytic infiltrate was noted infiltrating the mucosa. These lymphocytes had small cleaved-like nuclei with dense nuclear chromatin. First panel of immunostain was put and cells were positive for CD20, however diffuse positivity of the same cells with CD3 could not be ignored. Then more immunostains were performed. CD4 stained more lymphocytes than CD8. CD56 and CD30 were negative. However, CD20 expression on neoplastic cells limited the diagnosis of T-cell lymphoma. This case was subjected to T- and B-cell gene rearrangement. T- and B-cell gene rearrangement was done on the paraffin block. T-cell gene rearrangement study was positive and B-cell gene rearrangement study was negative. Morphology, immunostains and positive T-cell gene rearrangement analysis findings were consistent with enteropathy-associated T-cell lymphoma (EATL).
Figure 1: Duodenal, biopsy showing dense atypical lymphocytic infiltrate and blunting of villi (H and E, original magnifications ×10) (a). Enteropathy-associated T-cell lymphoma. immunohistochemistry for CD20 (b), CD3 (c), CD4 (d), and CD8 (e)

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Enteropathy-associated T-cell lymphoma is a very rare T-cell lymphoma that accounts for <5% of all gastrointestinal tract lymphomas. EATL most commonly arises in the proximal jejunum and less frequently elsewhere in the small intestine, stomach or colon. [1] EATL consists of two types of disorders: Type I EATL, which is a complication of celiac disease and the less frequent, type II EATL which is unrelated to celiac sprue. Both type I and type II EATL have poor clinical outcomes with a reported 5-year survival of 8-20%. [2]

Enteropathy-associated T-cell lymphoma shows a striking association with celiac disease, suggesting that chronic stimulation of intraepithelial lymphocytes by nutritional gluten may be an important factor in lymphomagenesis. EATL and celiac disease share clinical features and the characteristic pattern of gut involvement. Furthermore, EATL and celiac disease arise in patients with similar human leukocyte antigen haplotypes. [1]

Presence of significant CD20 positivity in our case obscured the true nature of lymphoma till the gene arrangement studies were done. Positivity for B-cell markers has been reported in nonB-cell lymphomas in earlier studies as well. Blakolmer et al. [3] investigated 94 extranodal nonB-cell lymphoma cases including 52 enteropathy-type intestinal T-cell lymphomas. Four of these cases, including three cases of enteropathy-type intestinal T-cell lymphoma and one of NK/T-cell lymphoma, expressed both T- and B-cell-specific surface markers. Another series by Rahemtullah et al. [4] reported nine cases of CD20 positive T-cell lymphoma out of which only one case was EATL.

Quintanilla-Martinez et al. [5] concluded that the CD20 positive T-cell lymphoma phenotype was not aberrant, but instead represented the neoplastic counterpart of a small subpopulation of normal peripheral blood T-cells expressing CD20.

Enteropathy-associated T-cell lymphoma accounts for fewer than 5% of all gastrointestinal lymphomas, however CD20 positive EATL are very rare with less than five cases reported in the literature. T-cell receptor rearrangement analysis is mandatory to nail down the diagnosis in such cases.

   References Top

Zettl A, deLeeuw R, Haralambieva E, Mueller-Hermelink HK. Enteropathy-type T-cell lymphoma. Am J Clin Pathol 2007;127:701-6.  Back to cited text no. 1
Van de Water JM, Cillessen SA, Visser OJ, Verbeek WH, Meijer CJ, Mulder CJ. Enteropathy associated T-cell lymphoma and its precursor lesions. Best Pract Res Clin Gastroenterol 2010;24:43-56.  Back to cited text no. 2
Blakolmer K, Vesely M, Kummer JA, Jurecka W, Mannhalter C, Chott A. Immunoreactivity of B-cell markers (CD79a, L26) in rare cases of extranodal cytotoxic peripheral T- (NK/T-) cell lymphomas. Mod Pathol 2000;13:766-72.  Back to cited text no. 3
Rahemtullah A, Longtine JA, Harris NL, Dorn M, Zembowicz A, Quintanilla-Fend L, et al. CD20+ T-cell lymphoma: Clinicopathologic analysis of 9 cases and a review of the literature. Am J Surg Pathol 2008;32:1593-607.  Back to cited text no. 4
Quintanilla-Martinez L, Preffer F, Rubin D, Ferry JA, Harris NL. CD20+ T-cell lymphoma. Neoplastic transformation of a normal T-cell subset. Am J Clin Pathol 1994;102:483-9.  Back to cited text no. 5

Correspondence Address:
Dr. Deepti Shukla Misra
Department of Histopathology, BLK Hospital, Pusa Road, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.142727

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