A Case Report of Monomorphic Epithelial Intestinal T-Cell Lymphoma and Literature Review

Jing  Deng; Yongguang  Zou; Guangshu  Li

doi:10.4236/ijcm.2020.112002

Jing Deng¹, Yongguang Zou¹^*, Guangshu Li²

Abstract: Background: Monomorphic epithelial intestinal T-cell lymphoma (MEITL), previously known as type II Enteropathy-associated T-cell lymphoma (EATL), is a rare intestinal tumor with strong invasiveness, poor prognosis, atypical clinical manifestations, and is difficult to diagnose. Aim: The clinical manifestations, imaging, histopathology, and immunohistochemical characteristics, treatment and prognosis of this case of MEITL were analyzed retrospectively, and combined with literatures learning to provide the experiences and lessons for diagnosis and treatment. Case Presentation: Here, we present a 68-year-old Asian woman with unexplained intestinal perforation and lung imaging changes as the first manifestation, and gradually appearing refractory diarrhea and pulmonary cavities. During the period, due to the unknown diagnosis, all treatments were mainly based on anti-infection and antidiarrhea, but her symptoms had no improvement after therapy, even died of poor basic physical conditions. Colonoscopy biopsy showed atypical lymphocyte infiltration-like growth and the formation of local superficial ulcers. Immunohistochemical tests showed that T-lymphocyte hyperplasia was predominant and led to a diagnosis of MEITL. Conclusion: MEITL is mainly manifested by gastrointestinal symptoms and lacks specific symptoms. The diagnosis of MEITL should be based on comprehensive judgment of clinical manifestations, pathological features and immunohistochemical detection results. Positive biopsy confirmed and timely chemotherapy may be conductive to a better prognosis.

Keywords: MEITL, Pulmonary Cavities, Refractory Diarrhea, Intestinal Perforation

Cite this paper: Deng, J. , Zou, Y. and Li, G. (2020) A Case Report of Monomorphic Epithelial Intestinal T-Cell Lymphoma and Literature Review. International Journal of Clinical Medicine, 11, 15-22. doi: 10.4236/ijcm.2020.112002.

References

[1] Song, M.J., Park, C.S., Hwang, H.S., et al. (2014) A Case of Type II Enteropathy-Associated T-Cell Lymphoma with Epstein-Barr Virus Positivity. Korean Journal of Pathology, 48, 426.
https://doi.org/10.4132/KoreanJPathol.2014.48.6.426

[2] Sakhdari, A. and Medeiros, L.J. (2019) Update on Lymphoma Classification: The 2016 Revised World Health Organization Classification of Hematopoietic and Lymphoid Neoplasms. Advances in Cell and Gene Therapy, 2, e57.
https://doi.org/10.1002/acg2.57

[3] Aiempanakit, K., Amatawet, C., Chiratikarnwong, K., et al. (2017) Erythema Multiforme-Like Cutaneous Lesions in Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma: A Rare Case Report. Journal of Cutaneous Pathology, 44, 183-188.
https://doi.org/10.1111/cup.12864

[4] Wang, L., Huang, D.H., Xiao, C.Y., et al. (2018) Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma: A Clinic-Pathologic Study of 3 Cases and Literature Review. Medical Journal of Chinese People’s Liberation Army, 43, 45-50.

[5] Antoniadou, F., Dimitrakopoulou, A., Voutsinas, P.M., et al. (2017) Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma in Pleural Effusion: A Case Report. Diagnostic Cytopathology, 45, 1050-1054.
https://doi.org/10.1002/dc.23772

[6] Kubota, Y. and Kusaba, K. (2018) Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma Involving the Central Nervous System. Blood, 131, 1765-1765.
https://doi.org/10.1182/blood-2017-11-819136

[7] Kato, A., Takiuchi, Y., Aoki, K., et al. (2011) Enteropathy-Associated T-Cell Lymphoma Type II Complicated by Autoimmune Hemolytic Anemia. Journal of Clinical and Experimental Hematopathology, 51, 119-123.
https://doi.org/10.3960/jslrt.51.119

[8] Samuel, R., Krill, T. and Merwat, S. (2019) Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma: A Rare Cause of Chronic Diarrhea. Journal of Gastrointestinal Cancer, 50, 1051-1054.
https://doi.org/10.1007/s12029-019-00210-3

[9] Jiao, G.H., Zheng, Z.Q. and Jiang, K. (2014) Enteropathy-Associated T-Cell Lymphoma Presenting with Gastrointestinal Tract Symptoms: A Report of Two Cases and Review of Diagnostic Challenges and Clinicopathological Correlation. Oncology Letters, 8, 91-94.
https://doi.org/10.3892/ol.2014.2105

[10] Gadkowski, L.B. and Stout, J.E. (2008) Cavitary Pulmonary Disease. Clinical Microbiology Reviews, 21, 305-333.
https://doi.org/10.1128/CMR.00060-07

[11] Roberti, A., Dobay, M.P., Bisig, B., et al. (2016) Type II Enteropathy-Associated T-Cell Lymphoma Features a Unique Genomic Profile with Highly Recurrent SETD2 Alterations. Nature Communications, 7, Article No. 12602.
https://doi.org/10.1038/ncomms12602

[12] Wang, Y.N., Li, J., Ni, Y.H., et al. (2018) The Clinical Characteristics of Patients with Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma Characterized by Minor Endoscopic Abnormalities. Chinese Journal of Internal Medicine, 57, 112-117.

[13] Wang, L., Chen, Y., Pan, X.Y., et al. (2011) Application of Ig/TCR Gene Rearrangement Analysis Combined with in Situ Hybridization with EBER in the Diagnosis of Gastrointestinal Lymphomas. Journal of Clinical & Experimental Pathology, 27, 580-585.

[14] Hong, Y.S., Woo, Y.S., Park, G., et al. (2016) Endoscopic Findings of Enteropathy-Associated T-Cell Lymphoma Type II: A Case Series. Gut Liver, 10, 147-151.
https://doi.org/10.5009/gnl14457

[15] Novakovic, B.J., Novakovic, S. and Frkovic-Grazio, S. (2006) A Single-Center Report on Clinical Features and Treatment Response in Patients with Intestinal T Cell Non-Hodgkin’s Lymphomas. Oncology Letters, 16, 191-195.
https://doi.org/10.3892/or.16.1.191

[16] Gentille, C., Qin, Q., Barbieri, A., et al. (2017) Use of PEG-Asparaginase in Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma, a Disease with Diagnostic and Therapeutic Challenges. Ecancer, 11, 771.
https://doi.org/10.3332/ecancer.2017.771

[17] Bishton, M.J. and Haynes, A.P. (2007) Combination Chemotherapy Followed by Autologoustem Cell Transplant for Enteropathy-Associated T Cell Lymphoma. British Journal of Haematology, 136, 111-113.
https://doi.org/10.1111/j.1365-2141.2006.06371.x