Intestinal Research 2017; 15(4): 546-547  http://dx.doi.org/10.5217/ir.2017.15.4.546
Monomorphic epitheliotropic intestinal T-cell lymphoma complicated by common variable immunodeficiency
Yang Liu1, Yue-hui Li1, Ji Li2, Jia-ming Qian2, Wei-xun Zhou3, and Ya-ping Luo4
1Department of Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China., 2Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China., 3Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China., 4Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Correspondence to: Ji Li, Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, No.1 Shuaifuyuan, Wangfujing, Dongcheng District, Beijing 100730, China. Tel: +86-10-60155019, Fax: +86-10-60155019, liji0235@pumch.cn
Received: August 1, 2017; Revised: August 2, 2017; Accepted: August 2, 2017; Published online: October 30, 2017.
© Korean Association for the Study of Intestinal Diseases. All rights reserved.

Question:

A 64-year-old man presented with chronic watery diarrhea and weight loss for 5 months prior to hospitalization. He had 8 to 15 bowel movements per day, with a total volume of 2 L. He had been in poor health for more than 30 years, mainly due to recurrent herpes zoster ophthalmicus, sinusitis, and upper respiratory tract infections. Physical examination was unremarkable except for a malnourished state with BMI of 14.8 kg/m2. Laboratory tests indicated low IgG and IgM levels (IgG, 372 mg/dL; IgM 21, mg/dL), and negative tissue transglutaminase and endomysial antibodies. Colonoscopy showed edematous but otherwise normal mucosa (Fig. A, white light) with mosaic-like pattern (Fig. B). Fluorodeoxyglucose-PET showed diffuse, intense radioactivity in the colon and rectum with standardized uptake value 2.6 to 5.1. Biopsy specimens were taken from the sigmoid and rectum. What is your diagnosis of this patient?

Answer to the Images: Monomorphic Epitheliotropic Intestinal T-cell Lymphoma in Combination with Common Variable Immunodeficiency

Biopsies revealed diffuse infiltration of monotonous, small-to-intermediate lymphocytes, with round or slightly irregular nuclei (Fig. C, ×100 and Fig. D, ×400; H&E). These atypical cells were immunochemically positive for CD3, CD7, CD8, and CD56, and negative for CD5 and EBV-encoded RNA, with a high Ki-67 index of 80%. The diagnosis of monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) was confirmed. Based on recurrent infection episodes for 30 years and hypoglobulinemia (IgG and IgM), common variable immunodeficiency (CVID) was clinically diagnosed. The patient refused chemotherapy and died 3 months later.

MEITL, or enteropathy-associated T-cell lymphoma, type II is a rare peripheral T-cell lymphoma commonly found in the small intestine. Endoscopic findings may include nodules, masses, strictures, and ulcers.1 Meanwhile, CVID is the most common primary immunodeficiency in adults, and gastrointestinal symptoms are commonly observed in CVID.2,3 Endoscopic findings depend on the underlying cause of the symptoms and are thus variable. In the long-term, patients with CVID are at higher risk of developing malignancies, especially non-Hodgkin's lymphoma.4 In the case of our patient, colonoscopy showed almost normal mucosa, which is unusual in MEITL, but fairly common in CVID. Therefore, biopsies should be performed even in the absence of obvious lesions. To our knowledge, this is the first reported case of CVID developing into MEITL.

References
  1. Ondrejka, S, and Jagadeesh, D (2016). Enteropathy-associated T-cell lymphoma. Curr Hematol Malig Rep. 11, 504-513.
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  2. Jørgensen, SF, Reims, HM, and Frydenlund, D (2016). A Cross-sectional study of the prevalence of gastrointestinal symptoms and pathology in patients with common variable immunodeficiency. Am J Gastroenterol. 111, 1467-1475.
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  3. Khodadad, A, Aghamohammadi, A, and Parvaneh, N (2007). Gastrointestinal manifestations in patients with common variable immunodeficiency. Dig Dis Sci. 52, 2977-2983.
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  4. Park, MA, Li, JT, Hagan, JB, Maddox, DE, and Abraham, RS (2008). Common variable immunodeficiency: a new look at an old disease. Lancet. 372, 489-502.
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