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Journal of Digestive Cancer Research 2023; 11(2): 114-118

Published online August 20, 2023

https://doi.org/10.52927/jdcr.2023.11.2.114

© Korean Society of Gastrointestinal Cancer Research

A Rare Case of Intestinal T-cell Lymphoma with Multiple Complications


Seung Yong Shin



Department of Internal Medicine, Chung-Ang University College of Medicine, Seoul, Korea

Correspondence to :
Seung Yong Shin
E-mail: mdthepage@gmail.com
https://orcid.org/0000-0001-8970-2444

Received: July 26, 2023; Accepted: August 7, 2023

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0). which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Intestinal T-cell lymphoma is a rare and aggressive form of non-Hodgkin lymphoma. Owing to its rarity and variable manifestations, intestinal T-cell lymphoma is usually diagnosed at an advanced stage. Therefore, it may be accompanied with serious complications at the time of diagnosis. Herein, I reports a case of intestinal T-cell lymphoma with multiple severe complications.

KeywordsLymphoma T-cell Intestine small Complications

The gastrointestinal (GI) tract is a common site for extranodal lymphoma involvement, accounting for 5–10% of all GI cancers [1]. However, the majority of primary GI lymphomas are of the B-cell lineage, with T-cell lymphomas accounting for only 4–9% [2-4]. Intestinal T-cell lymphoma tends to have various aggressive clinical manifestations [5,6], leading to severe complications. Here, I present a rare case of intestinal T-cell lymphoma with multiple complications.

A 70-year-old male presented with diffuse abdominal pain and weight loss of 4 kg over 3 months. The patient had no relevant medical history except for hypertension and dyslipidemia, and no history of surgery or family history. His vital signs were stable, and on physical examination, his abdomen was found to be soft but moderately distended without definite tenderness. Esophagogastroduodenoscopy and colonoscopy performed at another hospital one month prior showed no significant abnormalities. The patient’s laboratory data were as follows: white blood cell count, 18,860/mm3; hemoglobin, 15.9 g/dl; platelet count, 212,000/mm3. Abdominal computed tomography (CT) revealed multifocal segmentally enhanced wall thickening of the small bowel and homogenous enhancement of conglomerated lymph nodes (LNs) in the mesenteric root. An omental cake and a large amount of ascites were also identified (Fig. 1). Positron emission tomography showed diffuse/segmental wall thickening with moderate hypermetabolism in the ileum and several low-attenuated lesions with hypermetabolism in the enlarged spleen. Multiple hypermetabolic LNs were identified in the mesentery and retroperitoneum. For pathological diagnosis, laparoscopic partial omentectomy and ileal mesenteric LNs biopsy were performed. Hypovolemic shock developed on postoperative day 1, and a CT scan revealed spontaneous splenic rupture as the main cause; emergency splenectomy was performed to combat this complication (Fig. 2A). Three days after splenectomy, the patient developed hematochezia, leading to hypovolemic shock. CT tomography revealed small bowel bleeding in the ileum, for which emergency surgery was performed. The main small bowel lesion was identified 60 cm from the ileocecal valve and resected (Fig. 2B). Pathological examination revealed a segment of ileum with atrophic villi and transmural involvement of densely infiltrated lymphoma cells, which are relatively monomorphic small and round (Fig. 3). Immunohistochemical staining revealed that tumor cells are CD3+ and CD8+ (Fig. 4). Histological examination confirmed T-cell lymphoma as the cause of splenic rupture, and bone marrow examination further confirmed its presence. The final diagnosis was intestinal T-cell lymphoma, suspected to be monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL). The patient was referred to an oncologist and started on a regimen of cyclophosphamide, hydroxydaunorubicin, oncovin, and prednisolone (CHOP). However, five days after the first cycle of CHOP therapy with 50% dose reduction due to a recent surgery, the patient complained of severe abdominal pain, and a CT scan showed small-bowel perforation. Emergency small bowel resection was performed, and pathological examination revealed transmural involvement of intestinal T-cell lymphoma. The patient is currently receiving CHOP therapy.

Fig. 1.An abdominal computed tomography scan shows multifocal segmentally enhanced wall thickening of the small bowel, omental cake, and a large amount of ascites. (A) Transverse view image. (B) Coronal view image. Positron emission tomography shows (C) moderate hypermetabolism in the ileum and (D) several low-attenuated lesions with hypermetabolism in the enlarged spleen.

Fig. 2.Macroscopic finding of (A) spontaneously ruptured spleen and (B) main lesion of small bowel T-cell lymphoma leading to massive hematochezia.

Fig. 3.Microscopic findings of (A) a segment of ileum with transmural involvement of lymphoma cell (H&E, ×5), (B) atrophic villi and densely infiltrated lymphoma cells, which are relatively monomorphic small round. A few intraepithelial lymphoma cells are seen (H&E, ×400).

Fig. 4.Immunohistochemical stains. (A) CD3 positive, (B) CD8 positive, (C) CD56 negative, and (D) Ki-67 high proliferative activity (×100).

Intestinal T-cell lymphoma is a rare and aggressive form of non-Hodgkin lymphoma primarily affecting the GI tract [7]. It may involve different parts of the GI tract; however, the stomach is the most commonly affected site, while the small bowel is the second most common [8]. A recent World Health Organization classification system, revised in 2017, classified intestinal T-cell lymphoma into four types: enteropathy-associated T-cell lymphoma (EATL), MEITL, intestinal T-cell lymphoma not otherwise specified, and indolent T-cell lymphoproliferative disorder of the GI tract [9]. EATL is the most common subtype of intestinal T-cell lymphoma, more commonly observed in Europe and closely associated with celiac disease. In contrast, MEITL has no apparent association with celiac disease, and its incidence is higher in Asian and Hispanic populations and among men and middle-aged individuals [10,11]. The small intestine, especially the jejunum, is the most common site of MEITL [12].

The clinical presentation of intestinal T-cell lymphoma varies considerably between studies; however, common symptoms include abdominal pain, bloating, diarrhea, weight loss, and fatigue [7,13]. These symptoms may mimic other GI conditions, leading to a delayed diagnosis. As EATL is associated with celiac disease, it is often preceded by symptoms such as diarrhea and malabsorption, which are not usually detected in MEITL [14,15]. In addition, owing to its aggressive nature, intestinal T-cell lymphoma can quickly spread to other organs, such as the liver, spleen, and lymph nodes. Because disease progression can lead to severe complications, including intestinal obstruction or perforation, it is often diagnosed incidentally through the occurrence of these complications [13]. In the current case, the patient presented with abdominal pain and subsequently experienced spontaneous splenic rupture, GI hemorrhage, and small bowel perforation.

Diagnosis of intestinal T-cell lymphoma requires a combination of clinical evaluations, imaging studies, and laboratory tests. Endoscopic procedures, such as upper GI endoscopy and colonoscopy, are often performed to obtain biopsy samples from the affected intestinal area. These samples are then examined under a microscope to confirm the presence of abnormal T cells and to determine the specific lymphoma subtype. However, a previous multicenter prospective study reported that only 20% of intestinal T-cell lymphoma cases were diagnosed endoscopically [13]. In addition, endoscopically obtained tissues are often insufficient to accurately diagnose intestinal T-cell lymphoma. Moreover, its rarity also contributes to the difficulty of obtaining an accurate diagnosis. Consequently, patients are sometimes misdiagnosed with CD, intestinal tuberculosis, or cancer [6]. Immunophenotyping may help diagnose intestinal T-cell lymphoma and differentiate between disease subtypes. CD3+, CD4–, and CD5+ cells are typically identified in both EATL and MEITL. Additionally, MEITL cells can display CD8+, CD 56+, and CD 30– phenotypes [7,10].

The prognosis of intestinal T-cell lymphoma is generally poor, primarily because the disease is generally diagnosed at an advanced stage and displays aggressive behavior. Patients with MEITL often present with advanced-stage disease at the time of diagnosis, and the median overall survival of these patients has been reported as 14.8 months [10,16]. The 5-year survival rate for EATL has been estimated at 20% or less [13,17,18]. The treatment of intestinal T-cell lymphoma has proven challenging owing to its aggressive nature and the lack of standardized therapeutic approaches; currently, chemotherapy is the primary treatment for this condition. CHOP therapy has been widely adopted as an alternative treatment strategy; however, the response to treatment is generally poor [13,19,20]. Surgery is not recommended as a standard treatment, but is often performed for the diagnosis, treatment, and prevention of disease complications [6].

In conclusion, I reported a case of intestinal T-cell lymphoma with multiple complications. Intestinal T-cell lymphoma is often associated with severe complications; however, its rarity, variable presentation, and clinical course make diagnosis challenging. Clinical awareness and collaboration between gastroenterologists and oncologists are essential for timely diagnosis and appropriate management of the disease.

Heon-Kyun Ha, M.D. (Surgeon) and Soo Kee Min, M.D. (Pathologist) contributed to this report.

No potential conflict of interest relevant to this article was reported.

  1. Radaszkiewicz T, Dragosics B, Bauer P. Gastrointestinal malignant lymphomas of the mucosa-associated lymphoid tissue: factors relevant to prognosis. Gastroenterology 1992;102:1628-1638. https://doi.org/10.1016/0016-5085(92)91723-h.
    Pubmed CrossRef
  2. Shirwaikar Thomas A, Schwartz M, Quigley E. Gastrointestinal lymphoma: the new mimic. BMJ Open Gastroenterol 2019;6:e000320. https://doi.org/10.1136/bmjgast-2019-000320.
    Pubmed KoreaMed CrossRef
  3. Kohno S, Ohshima K, Yoneda S, Kodama T, Shirakusa T, Kikuchi M. Clinicopathological analysis of 143 primary malignant lymphomas in the small and large intestines based on the new WHO classification. Histopathology 2003;43:135-143. https://doi.org/10.1046/j.1365-2559.2003.01659.x.
    Pubmed CrossRef
  4. Kim SJ, Choi CW, Mun YC, et al. Multicenter retrospective analysis of 581 patients with primary intestinal non-hodgkin lymphoma from the Consortium for Improving Survival of Lymphoma (CISL). BMC Cancer 2011;11:321. https://doi.org/10.1186/1471-2407-11-321.
    Pubmed KoreaMed CrossRef
  5. Chun HB, Baek IH, Lee MS, et al. Jejunocolic fistula associated with an intestinal T cell lymphoma. Gut Liver 2011;5:387-390. https://doi.org/10.5009/gnl.2011.5.3.387.
    Pubmed KoreaMed CrossRef
  6. Sun ZH, Zhou HM, Song GX, Zhou ZX, Bai L. Intestinal T-cell lymphomas: a retrospective analysis of 68 cases in China. World J Gastroenterol 2014;20:296-302. https://doi.org/10.3748/wjg.v20.i1.296.
    Pubmed KoreaMed CrossRef
  7. Nishimura MF, Nishimura Y, Nishikori A, Yoshino T, Sato Y. Primary gastrointestinal T-cell lymphoma and indolent lymphoproliferative disorders: practical diagnostic and treatment approaches. Cancers (Basel) 2021;13:5774. https://doi.org/10.3390/cancers13225774.
    Pubmed KoreaMed CrossRef
  8. Bautista-Quach MA, Ake CD, Chen M, Wang J. Gastrointestinal lymphomas: morphology, immunophenotype and molecular features. J Gastrointest Oncol 2012;3:209-225. https://doi.org/10.3978/j.issn.2078-6891.2012.024.
    Pubmed KoreaMed CrossRef
  9. Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 2016;127:2375-2390. https://doi.org/10.1182/blood-2016-01-643569.
    Pubmed KoreaMed CrossRef
  10. Chan JK, Chan AC, Cheuk W, et al. Type II enteropathy-associated T-cell lymphoma: a distinct aggressive lymphoma with frequent γδ T-cell receptor expression. Am J Surg Pathol 2011;35:1557-1569. https://doi.org/10.1097/PAS.0b013e318222dfcd.
    Pubmed CrossRef
  11. Tse E, Gill H, Loong F, et al. Type II enteropathy-associated T-cell lymphoma: a multicenter analysis from the Asia Lymphoma Study Group. Am J Hematol 2012;87:663-668. https://doi.org/10.1002/ajh.23213.
    Pubmed CrossRef
  12. Tan SY, Chuang SS, Tang T, et al. Type II EATL (epitheliotropic intestinal T-cell lymphoma): a neoplasm of intra-epithelial T-cells with predominant CD8αα phenotype. Leukemia 2013;27:1688-1696. https://doi.org/10.1038/leu.2013.41.
    Pubmed CrossRef
  13. Daum S, Ullrich R, Heise W, et al. Intestinal non-Hodgkin's lymphoma: a multicenter prospective clinical study from the German Study Group on Intestinal non-Hodgkin's Lymphoma. J Clin Oncol 2003;21:2740-2746. https://doi.org/10.1200/JCO.2003.06.026.
    Pubmed CrossRef
  14. Malamut G, Chandesris O, Verkarre V, et al. Enteropathy associated T cell lymphoma in celiac disease: a large retrospective study. Dig Liver Dis 2013;45:377-384. https://doi.org/10.1016/j.dld.2012.12.001.
    Pubmed KoreaMed CrossRef
  15. Cellier C, Delabesse E, Helmer C, et al. Refractory sprue, coeliac disease, and enteropathy-associated T-cell lymphoma. French Coeliac Disease Study Group. Lancet 2000;356:203-208. https://doi.org/10.1016/s0140-6736(00)02481-8.
    Pubmed CrossRef
  16. Yi JH, Lee GW, Do YR, et al. Multicenter retrospective analysis of the clinicopathologic features of monomorphic epitheliotropic intestinal T-cell lymphoma. Ann Hematol 2019;98:2541-2550. https://doi.org/10.1007/s00277-019-03791-y.
    Pubmed CrossRef
  17. Novakovic BJ, Novakovic S, Frkovic-Grazio S. A single-center report on clinical features and treatment response in patients with intestinal T cell non-Hodgkin's lymphomas. Oncol Rep 2006;16:191-195. https://doi.org/10.3892/or.16.1.191.
    Pubmed CrossRef
  18. Nijeboer P, Malamut G, Mulder CJ, et al. Enteropathy-associated T-cell lymphoma: improving treatment strategies. Dig Dis 2015;33:231-235. https://doi.org/10.1159/000369542.
    Pubmed CrossRef
  19. Gale J, Simmonds PD, Mead GM, Sweetenham JW, Wright DH. Enteropathy-type intestinal T-cell lymphoma: clinical features and treatment of 31 patients in a single center. J Clin Oncol 2000;18:795-803. https://doi.org/10.1200/JCO.2000.18.4.795.
    Pubmed CrossRef
  20. Egan LJ, Walsh SV, Stevens FM, Connolly CE, Egan EL, McCarthy CF. Celiac-associated lymphoma. A single institution experience of 30 cases in the combination chemotherapy era. J Clin Gastroenterol 1995;21:123-129. https://doi.org/10.1097/00004836-199509000-00012.
    Pubmed CrossRef

Article

Case Report

Journal of Digestive Cancer Research 2023; 11(2): 114-118

Published online August 20, 2023 https://doi.org/10.52927/jdcr.2023.11.2.114

Copyright © Korean Society of Gastrointestinal Cancer Research.

A Rare Case of Intestinal T-cell Lymphoma with Multiple Complications

Seung Yong Shin

Department of Internal Medicine, Chung-Ang University College of Medicine, Seoul, Korea

Correspondence to:Seung Yong Shin
E-mail: mdthepage@gmail.com
https://orcid.org/0000-0001-8970-2444

Received: July 26, 2023; Accepted: August 7, 2023

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0). which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Intestinal T-cell lymphoma is a rare and aggressive form of non-Hodgkin lymphoma. Owing to its rarity and variable manifestations, intestinal T-cell lymphoma is usually diagnosed at an advanced stage. Therefore, it may be accompanied with serious complications at the time of diagnosis. Herein, I reports a case of intestinal T-cell lymphoma with multiple severe complications.

Keywords: Lymphoma, T-cell, Intestine, small, Complications

INTRODUCTION

The gastrointestinal (GI) tract is a common site for extranodal lymphoma involvement, accounting for 5–10% of all GI cancers [1]. However, the majority of primary GI lymphomas are of the B-cell lineage, with T-cell lymphomas accounting for only 4–9% [2-4]. Intestinal T-cell lymphoma tends to have various aggressive clinical manifestations [5,6], leading to severe complications. Here, I present a rare case of intestinal T-cell lymphoma with multiple complications.

CASE

A 70-year-old male presented with diffuse abdominal pain and weight loss of 4 kg over 3 months. The patient had no relevant medical history except for hypertension and dyslipidemia, and no history of surgery or family history. His vital signs were stable, and on physical examination, his abdomen was found to be soft but moderately distended without definite tenderness. Esophagogastroduodenoscopy and colonoscopy performed at another hospital one month prior showed no significant abnormalities. The patient’s laboratory data were as follows: white blood cell count, 18,860/mm3; hemoglobin, 15.9 g/dl; platelet count, 212,000/mm3. Abdominal computed tomography (CT) revealed multifocal segmentally enhanced wall thickening of the small bowel and homogenous enhancement of conglomerated lymph nodes (LNs) in the mesenteric root. An omental cake and a large amount of ascites were also identified (Fig. 1). Positron emission tomography showed diffuse/segmental wall thickening with moderate hypermetabolism in the ileum and several low-attenuated lesions with hypermetabolism in the enlarged spleen. Multiple hypermetabolic LNs were identified in the mesentery and retroperitoneum. For pathological diagnosis, laparoscopic partial omentectomy and ileal mesenteric LNs biopsy were performed. Hypovolemic shock developed on postoperative day 1, and a CT scan revealed spontaneous splenic rupture as the main cause; emergency splenectomy was performed to combat this complication (Fig. 2A). Three days after splenectomy, the patient developed hematochezia, leading to hypovolemic shock. CT tomography revealed small bowel bleeding in the ileum, for which emergency surgery was performed. The main small bowel lesion was identified 60 cm from the ileocecal valve and resected (Fig. 2B). Pathological examination revealed a segment of ileum with atrophic villi and transmural involvement of densely infiltrated lymphoma cells, which are relatively monomorphic small and round (Fig. 3). Immunohistochemical staining revealed that tumor cells are CD3+ and CD8+ (Fig. 4). Histological examination confirmed T-cell lymphoma as the cause of splenic rupture, and bone marrow examination further confirmed its presence. The final diagnosis was intestinal T-cell lymphoma, suspected to be monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL). The patient was referred to an oncologist and started on a regimen of cyclophosphamide, hydroxydaunorubicin, oncovin, and prednisolone (CHOP). However, five days after the first cycle of CHOP therapy with 50% dose reduction due to a recent surgery, the patient complained of severe abdominal pain, and a CT scan showed small-bowel perforation. Emergency small bowel resection was performed, and pathological examination revealed transmural involvement of intestinal T-cell lymphoma. The patient is currently receiving CHOP therapy.

Figure 1. An abdominal computed tomography scan shows multifocal segmentally enhanced wall thickening of the small bowel, omental cake, and a large amount of ascites. (A) Transverse view image. (B) Coronal view image. Positron emission tomography shows (C) moderate hypermetabolism in the ileum and (D) several low-attenuated lesions with hypermetabolism in the enlarged spleen.

Figure 2. Macroscopic finding of (A) spontaneously ruptured spleen and (B) main lesion of small bowel T-cell lymphoma leading to massive hematochezia.

Figure 3. Microscopic findings of (A) a segment of ileum with transmural involvement of lymphoma cell (H&E, ×5), (B) atrophic villi and densely infiltrated lymphoma cells, which are relatively monomorphic small round. A few intraepithelial lymphoma cells are seen (H&E, ×400).

Figure 4. Immunohistochemical stains. (A) CD3 positive, (B) CD8 positive, (C) CD56 negative, and (D) Ki-67 high proliferative activity (×100).

DISCUSSION

Intestinal T-cell lymphoma is a rare and aggressive form of non-Hodgkin lymphoma primarily affecting the GI tract [7]. It may involve different parts of the GI tract; however, the stomach is the most commonly affected site, while the small bowel is the second most common [8]. A recent World Health Organization classification system, revised in 2017, classified intestinal T-cell lymphoma into four types: enteropathy-associated T-cell lymphoma (EATL), MEITL, intestinal T-cell lymphoma not otherwise specified, and indolent T-cell lymphoproliferative disorder of the GI tract [9]. EATL is the most common subtype of intestinal T-cell lymphoma, more commonly observed in Europe and closely associated with celiac disease. In contrast, MEITL has no apparent association with celiac disease, and its incidence is higher in Asian and Hispanic populations and among men and middle-aged individuals [10,11]. The small intestine, especially the jejunum, is the most common site of MEITL [12].

The clinical presentation of intestinal T-cell lymphoma varies considerably between studies; however, common symptoms include abdominal pain, bloating, diarrhea, weight loss, and fatigue [7,13]. These symptoms may mimic other GI conditions, leading to a delayed diagnosis. As EATL is associated with celiac disease, it is often preceded by symptoms such as diarrhea and malabsorption, which are not usually detected in MEITL [14,15]. In addition, owing to its aggressive nature, intestinal T-cell lymphoma can quickly spread to other organs, such as the liver, spleen, and lymph nodes. Because disease progression can lead to severe complications, including intestinal obstruction or perforation, it is often diagnosed incidentally through the occurrence of these complications [13]. In the current case, the patient presented with abdominal pain and subsequently experienced spontaneous splenic rupture, GI hemorrhage, and small bowel perforation.

Diagnosis of intestinal T-cell lymphoma requires a combination of clinical evaluations, imaging studies, and laboratory tests. Endoscopic procedures, such as upper GI endoscopy and colonoscopy, are often performed to obtain biopsy samples from the affected intestinal area. These samples are then examined under a microscope to confirm the presence of abnormal T cells and to determine the specific lymphoma subtype. However, a previous multicenter prospective study reported that only 20% of intestinal T-cell lymphoma cases were diagnosed endoscopically [13]. In addition, endoscopically obtained tissues are often insufficient to accurately diagnose intestinal T-cell lymphoma. Moreover, its rarity also contributes to the difficulty of obtaining an accurate diagnosis. Consequently, patients are sometimes misdiagnosed with CD, intestinal tuberculosis, or cancer [6]. Immunophenotyping may help diagnose intestinal T-cell lymphoma and differentiate between disease subtypes. CD3+, CD4–, and CD5+ cells are typically identified in both EATL and MEITL. Additionally, MEITL cells can display CD8+, CD 56+, and CD 30– phenotypes [7,10].

The prognosis of intestinal T-cell lymphoma is generally poor, primarily because the disease is generally diagnosed at an advanced stage and displays aggressive behavior. Patients with MEITL often present with advanced-stage disease at the time of diagnosis, and the median overall survival of these patients has been reported as 14.8 months [10,16]. The 5-year survival rate for EATL has been estimated at 20% or less [13,17,18]. The treatment of intestinal T-cell lymphoma has proven challenging owing to its aggressive nature and the lack of standardized therapeutic approaches; currently, chemotherapy is the primary treatment for this condition. CHOP therapy has been widely adopted as an alternative treatment strategy; however, the response to treatment is generally poor [13,19,20]. Surgery is not recommended as a standard treatment, but is often performed for the diagnosis, treatment, and prevention of disease complications [6].

In conclusion, I reported a case of intestinal T-cell lymphoma with multiple complications. Intestinal T-cell lymphoma is often associated with severe complications; however, its rarity, variable presentation, and clinical course make diagnosis challenging. Clinical awareness and collaboration between gastroenterologists and oncologists are essential for timely diagnosis and appropriate management of the disease.

ACKNOWLEDGEMENTS

Heon-Kyun Ha, M.D. (Surgeon) and Soo Kee Min, M.D. (Pathologist) contributed to this report.

FUNDING

None.

CONFLICTS OF INTEREST

No potential conflict of interest relevant to this article was reported.

Fig 1.

Figure 1.An abdominal computed tomography scan shows multifocal segmentally enhanced wall thickening of the small bowel, omental cake, and a large amount of ascites. (A) Transverse view image. (B) Coronal view image. Positron emission tomography shows (C) moderate hypermetabolism in the ileum and (D) several low-attenuated lesions with hypermetabolism in the enlarged spleen.
Journal of Digestive Cancer Research 2023; 11: 114-118https://doi.org/10.52927/jdcr.2023.11.2.114

Fig 2.

Figure 2.Macroscopic finding of (A) spontaneously ruptured spleen and (B) main lesion of small bowel T-cell lymphoma leading to massive hematochezia.
Journal of Digestive Cancer Research 2023; 11: 114-118https://doi.org/10.52927/jdcr.2023.11.2.114

Fig 3.

Figure 3.Microscopic findings of (A) a segment of ileum with transmural involvement of lymphoma cell (H&E, ×5), (B) atrophic villi and densely infiltrated lymphoma cells, which are relatively monomorphic small round. A few intraepithelial lymphoma cells are seen (H&E, ×400).
Journal of Digestive Cancer Research 2023; 11: 114-118https://doi.org/10.52927/jdcr.2023.11.2.114

Fig 4.

Figure 4.Immunohistochemical stains. (A) CD3 positive, (B) CD8 positive, (C) CD56 negative, and (D) Ki-67 high proliferative activity (×100).
Journal of Digestive Cancer Research 2023; 11: 114-118https://doi.org/10.52927/jdcr.2023.11.2.114

References

  1. Radaszkiewicz T, Dragosics B, Bauer P. Gastrointestinal malignant lymphomas of the mucosa-associated lymphoid tissue: factors relevant to prognosis. Gastroenterology 1992;102:1628-1638. https://doi.org/10.1016/0016-5085(92)91723-h.
    Pubmed CrossRef
  2. Shirwaikar Thomas A, Schwartz M, Quigley E. Gastrointestinal lymphoma: the new mimic. BMJ Open Gastroenterol 2019;6:e000320. https://doi.org/10.1136/bmjgast-2019-000320.
    Pubmed KoreaMed CrossRef
  3. Kohno S, Ohshima K, Yoneda S, Kodama T, Shirakusa T, Kikuchi M. Clinicopathological analysis of 143 primary malignant lymphomas in the small and large intestines based on the new WHO classification. Histopathology 2003;43:135-143. https://doi.org/10.1046/j.1365-2559.2003.01659.x.
    Pubmed CrossRef
  4. Kim SJ, Choi CW, Mun YC, et al. Multicenter retrospective analysis of 581 patients with primary intestinal non-hodgkin lymphoma from the Consortium for Improving Survival of Lymphoma (CISL). BMC Cancer 2011;11:321. https://doi.org/10.1186/1471-2407-11-321.
    Pubmed KoreaMed CrossRef
  5. Chun HB, Baek IH, Lee MS, et al. Jejunocolic fistula associated with an intestinal T cell lymphoma. Gut Liver 2011;5:387-390. https://doi.org/10.5009/gnl.2011.5.3.387.
    Pubmed KoreaMed CrossRef
  6. Sun ZH, Zhou HM, Song GX, Zhou ZX, Bai L. Intestinal T-cell lymphomas: a retrospective analysis of 68 cases in China. World J Gastroenterol 2014;20:296-302. https://doi.org/10.3748/wjg.v20.i1.296.
    Pubmed KoreaMed CrossRef
  7. Nishimura MF, Nishimura Y, Nishikori A, Yoshino T, Sato Y. Primary gastrointestinal T-cell lymphoma and indolent lymphoproliferative disorders: practical diagnostic and treatment approaches. Cancers (Basel) 2021;13:5774. https://doi.org/10.3390/cancers13225774.
    Pubmed KoreaMed CrossRef
  8. Bautista-Quach MA, Ake CD, Chen M, Wang J. Gastrointestinal lymphomas: morphology, immunophenotype and molecular features. J Gastrointest Oncol 2012;3:209-225. https://doi.org/10.3978/j.issn.2078-6891.2012.024.
    Pubmed KoreaMed CrossRef
  9. Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 2016;127:2375-2390. https://doi.org/10.1182/blood-2016-01-643569.
    Pubmed KoreaMed CrossRef
  10. Chan JK, Chan AC, Cheuk W, et al. Type II enteropathy-associated T-cell lymphoma: a distinct aggressive lymphoma with frequent γδ T-cell receptor expression. Am J Surg Pathol 2011;35:1557-1569. https://doi.org/10.1097/PAS.0b013e318222dfcd.
    Pubmed CrossRef
  11. Tse E, Gill H, Loong F, et al. Type II enteropathy-associated T-cell lymphoma: a multicenter analysis from the Asia Lymphoma Study Group. Am J Hematol 2012;87:663-668. https://doi.org/10.1002/ajh.23213.
    Pubmed CrossRef
  12. Tan SY, Chuang SS, Tang T, et al. Type II EATL (epitheliotropic intestinal T-cell lymphoma): a neoplasm of intra-epithelial T-cells with predominant CD8αα phenotype. Leukemia 2013;27:1688-1696. https://doi.org/10.1038/leu.2013.41.
    Pubmed CrossRef
  13. Daum S, Ullrich R, Heise W, et al. Intestinal non-Hodgkin's lymphoma: a multicenter prospective clinical study from the German Study Group on Intestinal non-Hodgkin's Lymphoma. J Clin Oncol 2003;21:2740-2746. https://doi.org/10.1200/JCO.2003.06.026.
    Pubmed CrossRef
  14. Malamut G, Chandesris O, Verkarre V, et al. Enteropathy associated T cell lymphoma in celiac disease: a large retrospective study. Dig Liver Dis 2013;45:377-384. https://doi.org/10.1016/j.dld.2012.12.001.
    Pubmed KoreaMed CrossRef
  15. Cellier C, Delabesse E, Helmer C, et al. Refractory sprue, coeliac disease, and enteropathy-associated T-cell lymphoma. French Coeliac Disease Study Group. Lancet 2000;356:203-208. https://doi.org/10.1016/s0140-6736(00)02481-8.
    Pubmed CrossRef
  16. Yi JH, Lee GW, Do YR, et al. Multicenter retrospective analysis of the clinicopathologic features of monomorphic epitheliotropic intestinal T-cell lymphoma. Ann Hematol 2019;98:2541-2550. https://doi.org/10.1007/s00277-019-03791-y.
    Pubmed CrossRef
  17. Novakovic BJ, Novakovic S, Frkovic-Grazio S. A single-center report on clinical features and treatment response in patients with intestinal T cell non-Hodgkin's lymphomas. Oncol Rep 2006;16:191-195. https://doi.org/10.3892/or.16.1.191.
    Pubmed CrossRef
  18. Nijeboer P, Malamut G, Mulder CJ, et al. Enteropathy-associated T-cell lymphoma: improving treatment strategies. Dig Dis 2015;33:231-235. https://doi.org/10.1159/000369542.
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Journal Info

JDCR
Vol.12 No.1
April 20, 2024
eISSN : 2950-9505
pISSN : 2950-9394
Frequency: Triannual

open access

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Journal of Digestive Cancer Research

eISSN 2950-9505
pISSN 2950-9394

  • 2021
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  • 2024