Journal articles: 'Intraepithelial lymphoma'

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Rebollada-Merino, Agustín, Néstor Porras, Andrés Calvo-Ibbitson, Fernando Rodríguez-Franco, and Antonio Rodríguez-Bertos. "Bcl-2 Immunoexpression in Feline Epitheliotropic Intestinal T-Cell Lymphomas." Veterinary Sciences 9, no. 4 (March 31, 2022): 168. http://dx.doi.org/10.3390/vetsci9040168.

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Lymphoma is the most common malignant hematopoietic neoplasm in domestic felines. Twenty-two cases of feline epitheliotropic duodenal T-cell lymphoma were characterized morphologically and immunohistochemically (CD3, Pax5, Ki-67), and Bcl-2 immunoexpression was established. Most cases were in domestic shorthair cats (88.2%), with a mean age of 11.2 years. All lymphomas were CD3+, with a low-to-moderate expression of Ki-67 (<30%). A correlation between the tumoral pattern of infiltration in the lamina propria and the intraepithelial distribution of the neoplastic lymphocytes was established (p = 0.0155). Intraepithelial nests of neoplastic lymphocytes were predominantly observed in lymphomas with a patchy distribution in the lamina propria, whereas intraepithelial plaques were seen in lymphomas with an obliteration pattern. Bcl-2 was expressed in neoplastic cells in all cases, and a higher expression was associated with increased villous stunting (p = 0.0221), and tended to be present in those cases with increased epithelial damage. The expression of Bcl-2 and the degree of epitheliotropism were correlated with neoplastic progression in epitheliotropic intestinal T-cell lymphomas; those displaying high Bcl-2 immunoexpression showed increased villous stunting and epithelial damage, suggesting that Bcl-2 is overexpressed in advanced tumor stages, and may be used as a predictor of tumoral behavior in feline epitheliotropic intestinal T-cell lymphomas. This entity showed many similarities with human MEITL, so the latter entity should be considered in further lymphoma classifications of domestic animals.

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Attygalle, Ayoma D., Hongxiang Liu, Sima Shirali, Timothy C. Diss, Christoph Loddenkemper, Harald Stein, Ahmet Dogan, Ming-Qing Du, and Peter G. Isaacson. "Atypical marginal zone hyperplasia of mucosa-associated lymphoid tissue: a reactive condition of childhood showing immunoglobulin lambda light-chain restriction." Blood 104, no. 10 (November 15, 2004): 3343–48. http://dx.doi.org/10.1182/blood-2004-01-0385.

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Abstract Mucosa-associated lymphoid tissue (MALT) lymphomas usually arise at sites of acquired MALT and are uncommon in native MALT (eg, Peyer patches and tonsil). Malignancy in these low-grade lymphomas is often inferred by immunoglobulin light-chain restriction and expression of CD43; molecular genetic evidence is sought only if these are in doubt. We report 6 cases (4 tonsils, 2 appendixes) of marginal zone (MZ) hyperplasia in children aged 3 to 11 years that, despite histologic and immunophenotypic features indicative of lymphoma, were polyclonal by molecular analysis. No lymphoma-directed therapy was given and patients remain alive and well (5 cases, median follow-up 35.3 months). The involved tonsil and appendix showed florid MZ hyperplasia with prominent intraepithelial B cells (IEBCs). The MZ B cells and IEBCs showed a high-proliferation fraction and a CD20+, CD21+, CD27-, immunoglobulin (Ig) superfamily receptor translocation-associated 1-positive (IRTA-1+), CD43+, multiple myeloma oncogene 1 (MUM-1), IgM+D+ phenotype. Polymerase chain reaction (PCR), cloning, and sequencing of rearranged IgH and Igλ genes (whole tissue sections [6 cases]; microdissected cells [2 cases]) showed that the MZ B cells and IEBCs were polyclonal and the IgH genes nonmutated. In contrast, MZ (intraepithelial) B cells of 6 control tonsils had a similar immunophenotype, except for expression of CD27 and polytypic light chains, whereas molecular studies showed that they were polyclonal with mutated Ig genes. (Blood. 2004;104:3343-3348)

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Muram-Zborovski, Talia, Danielle Loeb, and Tsieh Sun. "Primary Intestinal Intraepithelial Natural Killer–like T-Cell Lymphoma: Case Report of a Distinct Clinicopathologic Entity." Archives of Pathology & Laboratory Medicine 133, no. 1 (January 1, 2009): 133–37. http://dx.doi.org/10.5858/133.1.133.

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Abstract Intestinal T-cell lymphoma is a heterogenous group. These tumors differ in their association with enteropathy, intraepithelial or nonintraepithelial origin, primary or secondary involvement, and T-cell or natural killer–like T-cell immunophenotype. There are also nonneoplastic conditions, such as celiac disease, refractory sprue, and reactive T-cell infiltration that mimic intestinal T-cell lymphoma. Therefore, the differential diagnosis requires extensive morphologic, immunophenotypic, and molecular genetic studies. A subset of primary intestinal intraepithelial T-cell lymphoma has emerged in recent years that is distinguished from enteropathy-type T-cell lymphoma in terms of clinical presentation (nonenteropathic), morphology (monomorphic small to medium-sized cells), immunophenotype (CD3−CD8+CD56+), and cytogenetics. We report such a case with a unique immunophenotype (CD3−, cytoplasmic CD3+, CD4−, CD8+, CD5−, CD7+, CD16−, CD56+, CD57−, CD103+, T-cell intracellular antigen 1+, and βF1+) that is comparable to that of a newly identified subset of intraepithelial T cells. The tumor progressed rapidly and the patient died within 6 months after the onset of the disease. We recommend a large monoclonal antibody panel for the differential diagnosis of this heterogeneous group of T-cell lymphoma.

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Heo, Mi Hwa, Hye Ra Jung, Young Rok Do, Jung-Sook Ha, Hyera Kim, Do-Hoon Kim, and Jin Young Kim. "Intraepithelial Lymphocytosis of the Resection Margin in the Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma." Blood 132, Supplement 1 (November 29, 2018): 5317. http://dx.doi.org/10.1182/blood-2018-99-115639.

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Abstract Background: Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a disease involving the intestine and is often diagnosed in surgically resected specimens. The disease prognosis of MEITL is fatal because of rapid recurrence and leakage after excision. Recently, in microscopic finding of MEITL, intraepithelial lymphocytosis in mucosa that were grossly intact has been reported. In this study, we evaluated the intraepithelial lymphocytosis of the resection margin to determine the reason for the early recurrence or leakage in MEITL. Materials and Methods: We reviewed the medical records of all patients who were diagnosed with small intestine lymphoma from January 1995 to June 2018 at our medical center. We analyzed a tissue array from 8 patients diagnosed with MEITL. The expression of CD3, CD5, and CD8 were analyzed by immunohistochemistry. Results: A total of 8 cases of small intestine lymphoma were collected in this period. There were three men (37.5%) and five women (mean age 55 years). Two patients died without initiation of chemotherapy. Six patients received chemotherapy after resection of MEITL (4 patients received CHOP regimens, 2 patients received ProMACE-CyBOM regimens), but five patients died within 6 months (mean survival time 4.1 months, range 0.3-12.1 months). Only one patient survived for 5 years. On gross examination, the distance to the resection margin was 7.58 cm (3.8-14.0 cm) on average. Microscopic examination revealed intraepithelial lymphocytosis at least of one of the resected margins in all case. Immunohistochemical staining for CD3, CD5, and CD8 was performed on the resection margins. As a result, severe intraepithelial lymphocytosis accompanied by aberrant loss of CD5 was observed in 6 cases (75%). Conclusion: In the MEITL, an intraepithelial lymphocytosis accompanied by aberrant CD5 loss is frequently observed in the resection margin, even if the resection margin is grossly intact, and there is no clear microscopic involvement of the lymphoma especially on low power. This fact is thought to be related to the early recurrence or leakage of MEITL at the surgical site. Disclosures No relevant conflicts of interest to declare.

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Yuan, Constance M., Steven Stein, John H. Glick, and Mariusz A. Wasik. "Natural Killer–like T-Cell Lymphoma of the Small Intestine With a Distinct Immunophenotype and Lack of Association With Gluten-Sensitive Enteropathy." Archives of Pathology & Laboratory Medicine 127, no. 3 (March 1, 2003): e142-e146. http://dx.doi.org/10.5858/2003-127-e142-nktlot.

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Abstract We report the case of a large natural killer (NK)–like T-cell lymphoma that involved the ileum and displayed a distinct immunophenotype and complex karyotype. The patient exhibited no evidence of gluten-sensitive enteropathy (celiac disease) or any other type of enteropathy as determined by clinical history, endoscopy, and serology for immunoglobulin A (IgA) antiendomysial and IgG antigliadin antibodies. Molecular studies demonstrated a clonal T-cell receptor γ chain gene rearrangement. Immunophenotype analysis showed expression of intestinal epithelium-homing receptor CD103, CD7, cytoplasmic CD3ɛ, CD56, and CD16 but no other T- or NK-cell markers. Cytogenetic analysis of the malignant cells revealed multiple chromosomal abnormalities indicative of a biologically advanced, high-grade lymphoma. A novel subset of normal intestinal intraepithelial lymphocytes, bearing a similar phenotype, has been described; moreover, this subset diminishes, rather than expands, in gluten-sensitive enteropathy. This case supports the notion that lymphomas involving the small intestine represent a heterogeneous group of lymphomas with diverse pathogenetic mechanisms.

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Kojima, Kazuhiro, James K. Chambers, Ko Nakashima, Yuko Goto-Koshino, and Kazuyuki Uchida. "Immunophenotyping of intraepithelial lymphocytes in canine chronic enteropathy and intestinal T-cell lymphoma using endoscopic samples." Veterinary Pathology 59, no. 2 (November 18, 2021): 227–35. http://dx.doi.org/10.1177/03009858211057220.

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Human enteropathy-associated T-cell lymphoma (EATL) is considered to be derived from intraepithelial lymphocytes (IELs); however, the origin of canine intestinal T-cell lymphoma (ITCL) remains unclear. Histological, immunohistochemical, and clonality examinations were performed using endoscopically collected canine duodenum samples of mucosal lesions of chronic enteropathy (CE; 73 cases) and ITCL without transmural neoplastic mass lesions (64 cases). Histopathological examinations revealed the intraepithelial accumulation of lymphocytes (called “intraepithelial lymphocytosis”) in 54/73 CE cases (74%) and the epitheliotropism of neoplastic lymphocytes in 63/64 ITCL cases (98%). Immunohistochemically, IELs in CE with intraepithelial lymphocytosis (IEL+CE) were diffusely immunopositive for CD3, with scattered immunopositivity for CD5, CD8, CD20, and granzyme B (GRB). The percentage of CD8+ in CD3+ IELs was significantly lower in IEL+CE than in CE without intraepithelial lymphocytosis (IEL−CE). Double-labeling immunohistochemistry revealed a high percentage of GRB expression in CD8− IEL among IEL+CE. Among 64 ITCL cases, CD3 was immunopositive in 64 (100%), CD5 in 22 (34%), CD8 in 8 (13%), CD20 in 12 (19%), CD30 in 13 (20%), and GRB in 49 (77%). In CD3+ cells, Ki67 immunopositivity was highest in ITCL, intermediate in IEL+CE, and lower in IEL−CE. A clonal TCR gene rearrangement was detected in 1/19 IEL−CE cases (5%), 15/54 IEL+CE (28%), and 38/58 ITCL (66%). These results indicate that the immunophenotype of canine ITCL (CD8−GRB+) is similar to that of the increased IELs in CE. The high proliferative activity and clonality of T cells in IEL+CE suggest that canine ITCL originates from these IELs, similar to human EATL.

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Nijeboer, Petula, Roy L. J. van Wanrooij, Greetje J. Tack, Chris J. J. Mulder, and Gerd Bouma. "Update on the Diagnosis and Management of Refractory Coeliac Disease." Gastroenterology Research and Practice 2013 (2013): 1–9. http://dx.doi.org/10.1155/2013/518483.

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A small subset of coeliac disease (CD) patients experiences persisting or recurring symptoms despite strict adherence to a gluten-free diet (GFD). When other causes of villous atrophy have been excluded, these patients are referred to as refractory celiac disease (RCD) patients. RCD can be divided in two types based on the absence (type I) or presence (type II) of an, usually clonal, intraepithelial lymphocyte population with aberrant phenotype. RCDI usually runs a benign course and may be difficult to be differentiated from uncomplicated, slow responding CD. In contrast, RCDII can be defined as low-grade intraepithelial lymphoma and frequently transforms into an aggressive enteropathy associated T-cell lymphoma with dismal prognosis. This paper describes the clinical characteristics of RCDI and RCDII, diagnostic approach, and the latest insights in treatment options.

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Hue, Susan Swee-Shan, Siok-Bian Ng, Shi Wang, and Soo-Yong Tan. "Cellular Origins and Pathogenesis of Gastrointestinal NK- and T-Cell Lymphoproliferative Disorders." Cancers 14, no. 10 (May 18, 2022): 2483. http://dx.doi.org/10.3390/cancers14102483.

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The intestinal immune system, which must ensure appropriate immune responses to both pathogens and commensal microflora, comprises innate lymphoid cells and various T-cell subsets, including intra-epithelial lymphocytes (IELs). An example of innate lymphoid cells is natural killer cells, which may be classified into tissue-resident, CD56bright NK-cells that serve a regulatory function and more mature, circulating CD56dim NK-cells with effector cytolytic properties. CD56bright NK-cells in the gastrointestinal tract give rise to indolent NK-cell enteropathy and lymphomatoid gastropathy, as well as the aggressive extranodal NK/T cell lymphoma, the latter following activation by EBV infection and neoplastic transformation. Conventional CD4+ TCRαβ+ and CD8αβ+ TCRαβ+ T-cells are located in the lamina propria and the intraepithelial compartment of intestinal mucosa as type ‘a’ IELs. They are the putative cells of origin for CD4+ and CD8+ indolent T-cell lymphoproliferative disorders of the gastrointestinal tract and intestinal T-cell lymphoma, NOS. In addition to such conventional T-cells, there are non-conventional T-cells in the intra-epithelial compartment that express CD8αα and innate lymphoid cells that lack TCRs. The central feature of type ‘b’ IELs is the expression of CD8αα homodimers, seen in monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), which primarily arises from both CD8αα+ TCRαβ+ and CD8αα+ TCRγδ+ IELs. EATL is the other epitheliotropic T-cell lymphoma in the GI tract, a subset of which arises from the expansion and reprograming of intracytoplasmic CD3+ innate lymphoid cells, driven by IL15 and mutations of the JAK-STAT pathway.

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de Baaij, Laura R., Marijn Radersma, Jolanda MW van de Water, Kim BJ Groot, Nathalie J. Hijmering, Laura M. Moesbergen, Otto J. Visser, et al. "Bortezomib Restores Defective Apoptosis by Upregulation of Noxa in Enteropathy-Associated T-Cell Lymphoma." Blood 118, no. 21 (November 18, 2011): 2722. http://dx.doi.org/10.1182/blood.v118.21.2722.2722.

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Abstract Abstract 2722 Enteropathy-associated T-cell lymphoma (EATL) is a rare intestinal lymphoma that arises from intraepithelial lymphocytes. Clinical outcome of patients with EATL is very poor, due to chemotherapy-resistance and high relapse rates. Therefore, new therapeutic options for EATL are urgently needed. Studies in other types of lymphoma have shown that inhibition of apoptosis may cause chemotherapy-resistance and that restoration of defective apoptosis can induce cell death in these lymphomas. Preliminary data in EATL samples have demonstrated an increased expression of a fraction of NF-κB target genes, suggesting upregulation of NF-κB activity in EATL tumor cells. NF-κB activity can be inhibited by the proteasome inhibitor bortezomib resulting in induction of apoptosis. In the present study, we evaluated if apoptosis is inhibited in EATL cells and if Bortezomib can restore apoptosis in EATL cells. Laser-capture microdissection was applied to 16 fresh frozen EATL samples to obtain purified tumor cells for RNA isolation. Intraepithelial lymphocytes (IEL) of healthy controls were obtained from fresh duodenal biopsies and isolated by cell sorting. RT-MLPA analysis revealed that the pro-apoptotic BH3-only gene Noxa was significantly downregulated in most EATL samples compared to healthy donor IEL. Induction with etoposide resulted in caspase-9 mediated apoptosis in EATL cells with relatively high Noxa expression, whereas in EATL cells with low Noxa expression no apoptosis was induced, suggesting an inhibition in the intrinsic apoptosis pathway. Treatment with Bortezomib resulted in induction of apoptosis in EATL cells. The lethal dose (LD50) varied between 7.5 nM and 15 nM. Bortezomib induced cell death in EATL cells was caspase-9 mediated. mRNA and protein expression analysis showed upregulation of Noxa after incubation with bortezomib. In conclusion, our study showed that bortezomib induces apoptosis by upregulation of Noxa in EATL cells. Bortezomib therefore may be a potential drug in the treatment of patients with EATL. Disclosures: No relevant conflicts of interest to declare.

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Tjon, Jennifer M. L., Wieke H. M. Verbeek, Yvonne M. C. Kooy-Winkelaar, Binh H. Nguyen, Arno R. van der Slik, Allan Thompson, Mirjam H. M. Heemskerk, et al. "Defective synthesis or association of T-cell receptor chains underlies loss of surface T-cell receptor–CD3 expression in enteropathy-associated T-cell lymphoma." Blood 112, no. 13 (December 15, 2008): 5103–10. http://dx.doi.org/10.1182/blood-2008-04-150748.

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Abstract Enteropathy-associated T-cell lymphoma, an often fatal complication of celiac disease, can result from expansion of aberrant intraepithelial lymphocytes in refractory celiac disease type II (RCD II). Aberrant intraepithelial lymphocytes and lymphoma cells are intracellularly CD3ϵ+ but lack expression of the T-cell receptor (TCR)–CD3 complex on the cell surface. It is unknown what causes the loss of TCR-CD3 expression. We report the isolation of a cell line from an RCD II patient with the characteristic phenotype of enteropathy-associated T-cell lymphoma. We demonstrate that in this cell line the TCR-α and -β chains as well as the CD3γ, CD3δ, CD3ϵ, and ζ-chains are present intracellularly and that assembly of the CD3γϵ, CD3δϵ, and ζζ-dimers is normal. However, dimerization of the TCR chains and proper assembly of the TCR-CD3 complex are defective. On introduction of exogenous TCR-β chains, but not of TCR-α chains, assembly and functional cell surface expression of the TCR-CD3 complex were restored. Defective synthesis of both TCR chains was found to underlie loss of TCR expression in similar cell lines isolated from 2 additional patients. (Pre)malignant transformation in RCD II thus correlates with defective synthesis or defective association of the TCR chains, resulting in loss of surface TCR-CD3 expression.

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Harbert, J. L., F. Da Silva Lameira, W. Beversdorf, R. Bhalla, and E. Rinker. "CD30+ Anaplastic Enteropathy-Associated T-cell Lymphoma Mimicking Crohn’s Disease." American Journal of Clinical Pathology 156, Supplement_1 (October 1, 2021): S95—S96. http://dx.doi.org/10.1093/ajcp/aqab191.203.

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Abstract Introduction/Objective Enteropathy-associated T-cell lymphoma (EATL) is an aggressive peripheral T-cell lymphoma with a very poor prognosis. It is not uncommon for patients with EATL to present with intestinal obstruction or perforation, with the diagnosis made following surgical resection or autopsy. Although EATL is associated with celiac disease, this diagnosis may be made concomitant with EATL. The symptomology is often non-specific, particularly without a known history of celiac disease, and progression may be rapid. Methods/Case Report We describe the case of a 57 year old male who presented with several months of diarrhea, fatigue, night sweats, severe weight loss, and failure to thrive. Initial workup indicated positivity for Clostridium difficile and cryptosporidium, leading to antibiotic treatment. His symptoms persisted without significant improvement and a presumptive diagnosis of Crohn’s disease was made based on colonoscopy with biopsy and imaging that showed inflammatory changes with entero-enteric fistula formation. Further clinical decline necessitated exploratory laparotomy which revealed multiple enteric strictures, intra-loop abscesses, and necrotic ulceration necessitating segmental ileal resections. There was no associated lymphadenopathy. Microscopically, there was a dense, polymorphic lymphoid population within the bowel wall with associated mucosal ulceration and abundant necrosis. Striking anaplastic cytomorphology was present, with a heavy intratumoral eosinophilic infiltrate. Neoplastic lymphocytes were CD3+, CD30+ T cells with a CD7+, CD5-, CD4-, CD8-, CD56-, ALK- immunophenotype. The adjacent intestinal mucosa showed features of celiac disease (villous atrophy, intraepithelial lymphocytosis). Results (if a Case Study enter NA) NA Conclusion The prominent anaplastic morphology in combination with strong, diffuse CD30 expression prompted consideration of other T cell lymphomas, including anaplastic large cell lymphoma and peripheral T cell lymphoma, NOS, within the differential. The presence of coexisting histologic changes of celiac disease, dense eosinophilic infiltrate, and absence of lymphadenopathy were clues to the correct diagnosis. Notably, the frequency of CD30 expression in EATL is variable but common among cases manifesting large cell morphology.

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Török, László, Péter Kozma, Lajos Kocsis, Enikő Bagdi, and László Krenács. "Primer indolent intraepithelial gamma/delta T-cell lymphoma of the oral mucosa." Bőrgyógyászati és Venerológiai Szemle 91, no. 4 (August 28, 2015): 144–46. http://dx.doi.org/10.7188/bvsz.2015.91.4.3.

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Hughes, RG, M. Colquhoun, DM Eccles, M. Alloub, AC Parker, M. Norval, and GE Smart. "Cervical intraepithelial neoplasia in lymphoma patients: a cytological and colposcopic study." British Journal of Cancer 59, no. 4 (April 1989): 594–99. http://dx.doi.org/10.1038/bjc.1989.120.

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Kamaleshwaran, Koramadai Karuppusamy, Ramkumar Elumalai, and Bharat Rangarajan. "Fluorine-18 Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography Uptake of Jejunal Lymphocytosis Due to Giardiasis Mimicking Lymphoma Recurrence." Indian Journal of Nuclear Medicine 39, no. 1 (2024): 59–60. http://dx.doi.org/10.4103/ijnm.ijnm_70_23.

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Giardia lamblia is an intestinal protozoan with oral-fecal transmission. Infection is predominantly located in the small intestine. Giardiasis causes intraepithelial lymphocytosis in the small intestine which causes false-positive fluorodeoxyglucose (FDG) uptake mimicking malignancy. We present here an interesting image of fluorine-18 FDG positron emission tomography/computed tomography showing jejunal uptake caused by giardiasis in a known case of lymphoma.

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Reddy, Vijaya Lakshmi Muram, Durga Kharidehal, and Syam Sundara Rao Byna. "An Unusual Occurrence of Immunoproliferative Small Intestinal Disease in Elderly Patient Presenting with Chronic Diarrhea - A Rare Case Report." International Journal of Research and Review 11, no. 2 (February 16, 2024): 6–10. http://dx.doi.org/10.52403/ijrr.20240202.

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Immunoproliferative small intestinal disease (IPSID) is a rare type of indolent B-cell lymphoma. It is a variant of extranodal marginal zone B-cell lymphoma of mucosa associated lymphoid tissue. It is commonly seen in older children and young adults in the age group of 10-35 years. It is more common in males with Male: Female ratio of 2.4:1. Duodenum and jejunum are the commonly involved areas in small intestine. Colicky abdominal pain and intermittent diarrhoea are the common symptoms. Here by, we report a rare case in a 74-year-old male patient presented with chief complaints of abdominal pain, diarrhea and weight loss for 3 months. CT scan findings revealed thickening of small intestinal wall and mesenteric lymphadenopathy. Upper gastrointestinal endoscopy findings revealed nodular and ulcerative mucosa in the duodenum. Histopathological examination revealed flattening of mucosa with villous atrophy, destruction of crypts and intraepithelial lymphocytic infiltrate is noted. Interstitium shows dense and diffuse collection of lymphoplasmacytic infiltrate. Immunohistochemistry was done and it showed CD20 positive lymphoid cells. Based on the above findings the case was reported as Immunoproliferative small intestinal disease. Clinicians should suspect IPSID in cases presenting with chronic diarrhea and abdominal pain refractory to treatment. Immunoproliferative small intestinal disease has indolent clinical course and it can be cured with antibiotics in early stages. Late stages have high mortality rate and has poor prognosis. Keywords: IPSID, Alphachain disease, B cell lymphoma, duodenum, villous atrophy, lymphoplasmacytic infiltrate

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Moser, Katharina, Erik Teske, Wolf Bomhard, Christian Stockhaus, and Stefanie Mitze. "Correlation between the FCEAI and diagnostic para meters in chronic enteropathies in 147 cats (2006–2012)." Tierärztliche Praxis Ausgabe K: Kleintiere / Heimtiere 45, no. 06 (2017): 390–96. http://dx.doi.org/10.15654/tpk-170089.

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SummaryObjective: The Feline Chronic Enteropathy Activity Index (FCEAI) has been established as a quantitative index for disease activity in chronic enteropathies in cats. A definite diagnosis is aimed at histology with initial exclusion of extraintestinal causes by laboratory examinations, diagnostic imaging and endoscopy. The study aimed to examine diagnostic parameters and FCEAI in chronic gastroenteropathies. Materials and methods: A retrospective case review of 147 cats with chronic enteropathies was performed. In all patients, the FCEAI was established and endoscopy performed including biopsies and duodenal cytology. Histopathologic reports were reviewed for the diagnosis of lymphoma and architectural changes (epithelial integrity, villi/ gland atrophy, intestinal crypt atrophy, lymphangiectasia, epitheliotropism/infiltration of intraepithelial lymphocytes). A cytopathologic score (CS) and histopathologic score (HS) regarding lymphocytic intestinal infiltration were assigned. Statistical dependency analysis was used to determine correlations between the FCEAI, lymphoma, architectural changes, CS, HS, serum concentrations of cobalamin, folate and albumin. Results: The 147 cats consisted of predominately European Shorthair cats (n = 126), were mostly castrated (n = 127) and had a mean age of 9.8 (1–17) years. For the proven lymphoma group (12.2%; n = 18) and the non-lymphoma group a mean FCEAI of 7.3 (4–17) and 6.6 (2–13), respectively, was established. The FCEAI showed a low correlation with the CS (p = 0.010; R = 0.22) and intestinal villous atrophy (n = 121; p = 0.035; R = 0.19). Cats with a CS of 0 had a significant lower FCEAI score (p = 0.015) than cats with all other CSs. The histo- and cytopathologic scores were highly related (p < 0.001; R = 0.43). The gastric intraepithelial lymphocytic infiltration (n = 131) was significantly correlated to serum folate (p = 0.014; R = –0.56) and albumin (p = 0,048; R = –0.20). Conclusion: The FCEAI showed only a few correlations. Not only the grade of inflammation, but also the histologic architectural changes are of importance.

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Van Beurden, Yvette H., Tom Van Gils, Nienke A. Van Gils, Zain Kassam, Chris J. J. Mulder, and Nieves Aparicio-Pagés. "Serendipity in Refractory Celiac Disease: Full Recovery of Duodenal Villi and Clinical Symptoms after Fecal Microbiota Transfer." Journal of Gastrointestinal and Liver Diseases 25, no. 3 (September 1, 2016): 385–88. http://dx.doi.org/10.15403/jgld.2014.1121.253.cel.

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Treatment of refractory celiac disease type II (RCD II) and preventing the development of an enteropathy associated T-cell lymphoma in these patients is still difficult. In this case report, we describe a patient with RCD II who received fecal microbiota transfer as treatment for a recurrent Clostridium difficile infection, and remarkably showed a full recovery of duodenal villi and disappearance of celiac symptoms. This case suggests that altering the gut microbiota may hold promise in improving the clinical and histological consequences of celiac disease and/or RCD II. Abbreviations: CDI: Clostridium difficile infection; EATL : enteropathy associated T-cell lymphoma; FMT: fecal microbiota transfer; IEL: intraepithelial lymphocytes; RCD II: refractory celiac disease type II; TPN: total parenteral nutrition.

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Nguyen, Thuong-Thuong, Matthew Gubens, Daniel A. Arber, Ranjana Advani, Margrit Juretzka, and Natali Aziz. "Lymphoma in Pregnancy Initially Diagnosed as Vaginal Intraepithelial Neoplasia and Lichen Planus." Obstetrics & Gynecology 118, no. 2, Part 2 (August 2011): 486–89. http://dx.doi.org/10.1097/aog.0b013e3182234d12.

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Chen, Qian, Heng-Hui Cheng, Shuang Deng, Dong Kuang, Chang Shu, Li Cao, Guang-Quan Liao, Qiao-Zhen Guo, and Qi Zhou. "Diagnosis of Superficial Gastric Lesions Together with Six Gastric Lymphoma Cases via Probe-Based Confocal Laser Endomicroscopy: A Retrospective Observational Study." Gastroenterology Research and Practice 2018 (June 13, 2018): 1–8. http://dx.doi.org/10.1155/2018/5073182.

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Objective. To evaluate the performance of probe-based confocal laser endomicroscopy (pCLE) in diagnosis of gastric lesions.Methods. An outpatient department- (OPD-) based retrospective study was conducted for patients with suspected upper gastrointestinal (GI) tract lesions who underwent pCLE between 2014 and 2016 at a tertiary hospital in China. Final diagnosis was based on the histopathological reports. CLE reports were compared to histopathological reports to evaluate the diagnostic ability, including sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and diagnostic accuracy.Results. 322 of 380 patients were diagnosed with gastric lesions via pCLE, including inflammation and benign ulcers (n=110), atrophy and intestinal metaplasia (n=152), intraepithelial neoplasia (n=27), adenocarcinoma (n=27), and lymphoma (n=6). In total, the diagnostic ability of CLE in evaluation of gastric lesions showed sensitivity 72.4% (95% confidence interval (CI): 67.1–77.2%); specificity 93.1% (95% CI: 5.6–8.4%); PPV 72.4% (95% CI: 67.1–77.2%); NPV 93.1% (95% CI: 5.6–8.4%); and accuracy 88.9% (95% CI: 87.3–90.4%), respectively. We further observed the capability of pCLE in diagnosing six gastric lymphoma showing those affected mucosa densely infiltrated with identical and round-shaped abnormal cells. Immunohistochemistry analysis confirmed one patient with diffuse large B-cell non-Hodgkin’s lymphoma (DLBCL) and five with mucosa-associated lymphoid tissue (MALT) lymphoma.Conclusion. pCLE is an accurate tool for the detection of gastric lesions and shows optimal values of sensitivity and negative predictivity. Moreover, combining pCLE with white light endoscopy (WLE) may be a promising adjunct to conventional biopsy sampling in evaluating GI tract with suspected lymphoma.

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Bagdi, Eniko, Timothy C. Diss, Philippa Munson, and Peter G. Isaacson. "Mucosal Intra-epithelial Lymphocytes in Enteropathy-Associated T-Cell Lymphoma, Ulcerative Jejunitis, and Refractory Celiac Disease Constitute a Neoplastic Population." Blood 94, no. 1 (July 1, 1999): 260–64. http://dx.doi.org/10.1182/blood.v94.1.260.413k40_260_264.

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Loss of response to a gluten-free diet (refractory sprue) and ulcerative jejunitis are complications of celiac disease that may progress to enteropathy-associated T-cell lymphoma (EATL). Both conditions are characterized by the presence of a nonlymphomatous monoclonal T-cell population in the enteropathic mucosa. In EATL, a similar monoclonal population that shows clonal identity with the lymphoma itself is also present in the enteropathic mucosa. In this study we show that in all three circumstances the monoclonal T-cell population is constituted by cytologically normal, noninvasive intraepithelial T lymphocytes that share an identical aberrant immunophenotype with EATL. Patients with refractory sprue and/or ulcerative jejunitis are, therefore, suffering from a neoplastic T-cell disorder for which hematological treatment strategies need to be devised.

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Di Sabatino, Antonio, Federico Biagi, Paolo G. Gobbi, and Gino R. Corazza. "How I treat enteropathy-associated T-cell lymphoma." Blood 119, no. 11 (March 15, 2012): 2458–68. http://dx.doi.org/10.1182/blood-2011-10-385559.

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Abstract Enteropathy-associated T-cell lymphoma (EATL) is a complication of celiac disease (CD). This tumor derives from the neoplastic transformation of aberrant intraepithelial T lymphocytes emerging in celiac patients unresponsive to a gluten-free diet. Poor adherence to a gluten-free diet, HLA-DQ2 homozygosity, and late diagnosis of CD are recognized as risk factors for malignant evolution of CD. Recurrence of diarrhea, unexplained weight loss, abdominal pain, fever, and night sweating should alert physicians to this complication. The suspicion of EATL should lead to an extensive diagnostic workup in which magnetic resonance enteroclysis, positron emission tomography scan, and histologic identification of lesions represent the best options. Treatment includes high-dose chemotherapy preceded by surgical resection and followed by autologous stem cell transplantation, although biologic therapies seem to be promising. Strict adherence to a gluten-free diet remains the only way to prevent EATL.

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Tomita, Sakura, Yara Yukie Kikuti, Joaquim Carreras, Rika Sakai, Katsuyoshi Takata, Tadashi Yoshino, Silvia Bea, et al. "Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma in Asia Frequently Shows SETD2 Alterations." Cancers 12, no. 12 (November 27, 2020): 3539. http://dx.doi.org/10.3390/cancers12123539.

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Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare primary T-cell lymphoma of the digestive tract derived from intraepithelial lymphocytes and characterized by an aggressive clinical course. In this study, nine cases of Japanese MEITL were analyzed by targeted Next Generation Sequencing (NGS) and immunohistochemistry and were integrated with previously reported whole-genome copy number microarray-based assay data. The highlight of our findings is that all cases showed alterations of the tumor suppressor gene SETD2 by mutations and/or loss of the corresponding 3p21 locus. We also demonstrated that all cases showed mutations in one or more genes of JAK/STAT pathway. Therefore, the combination of epigenetic deregulation and cell signaling activation represent major oncogenic events in the pathogenesis of MEITL in Asian MEITL, similar to Western MEITL.

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Inagaki, Naoko, Daisuke Asaoka, Kiyoshi L. Mori, Naomi Sohda, Ichiro Miura, Hiroto Miwa, Nobuhiro Sato, and Kazuo Oshimi. "Enteropathy-Type T-Cell Lymphoma Expressing NK-Cell Intraepithelial Lymphocyte (NK-IEL) Phenotype." Leukemia & Lymphoma 45, no. 7 (July 2004): 1471–74. http://dx.doi.org/10.1080/10428190310001653709.

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Tsujikawa, Tomoyuki, Akihiko Itoh, Masamiti Bamba, Akira Andoh, Keiko Hodohara, Hisayuki Inoue, Yoshihide Fujiyama, and Tadao Bamba. "Aggressive jejunal γ δT-cell lymphoma derived from intraepithelial lymphocytes: An autopsy case report." Journal of Gastroenterology 33, no. 2 (March 10, 1998): 280–84. http://dx.doi.org/10.1007/s005350050084.

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25

Bacci, Barbara, Andrew William Stent, and Elizabeth Ann Walmsley. "Equine Intestinal Lymphoma: Clinical-Pathological Features, Immunophenotype, and Survival." Veterinary Pathology 57, no. 3 (March 23, 2020): 369–76. http://dx.doi.org/10.1177/0300985820906889.

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Lymphoma is the most common intestinal neoplasm in horses, but its clinical-pathological features are poorly characterized. Primary intestinal lymphoma was diagnosed in 20 horses on biopsy samples and further confirmed by postmortem examination in 16 cases. Lymphoma was found in the small intestine in 12 of 20 (60%), in the colon in 5 of 20 (25%), and in both small and large intestines in 3 of 20 (15%) cases. Gross findings included thickening of the intestinal wall (45%), mural nodules or masses (30%), and both thickening and nodules (10%). Cases were classified according to the human World Health Organization classification as enteropathy-associated T-cell lymphoma (EATL) type 1 (40%), EATL type 2 (45%), and T-cell-rich large B-cell lymphoma (TCRLBCL) (15%). With respect to histologic grade, 70% of cases were grade 1 and 30% were grade 2. Of EATLs, the infiltrate was mucosal only (12%), mucosal and submucosal (53%), or transmural (35%). EATL1 was submucosal to transmural (2/8 and 6/8), EATL2 was mucosal to submucosal (3/9 and 6/9), and TCRLBCL was always transmural. Epitheliotropism was present in most EATLs and characterized by single-cell infiltrates within the epithelium in EATL1 and intraepithelial clusters or plaques in EATL2. Median survival was 25 days for EATL1, 90 days for EATL2, and 187.5 days for TCRLBCL; differences were not statistically significant. Of the EATLs, grade 1 had a median survival of 60 days and grade 2 had a median survival of 25 days; differences were not statistically significant.

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C., Maier. "Rapidly progressive high-grade cervical intraepithelial neoplasia in a Hodgkin lymphoma patient: A Case Report." Gineco.eu 11, no. 3 (September 20, 2015): 111–13. http://dx.doi.org/10.18643/gieu.2015.111.

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27

Di Sabatino, A., R. Ciccocioppo, S. D'Aló, L. Ricevuti, F. Bonvicini, M. G. Cifone, and G. R. Corazza. "Lamina propria (LPL) and intraepithelial lymphocyte (IEL) apoptosis in coeliac disease-associated T-cell lymphoma." Digestive and Liver Disease 33 (November 2001): A66. http://dx.doi.org/10.1016/s1590-8658(01)80416-3.

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Wright, D. H., D. B. Jones, H. Clark, G. M. Mead, E. Hodges, and W. M. Howell. "Is adult-onset coeliac disease due to a low-grade lymphoma of intraepithelial T lymphocytes?" Lancet 337, no. 8754 (June 1991): 1373–74. http://dx.doi.org/10.1016/0140-6736(91)93059-i.

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29

Xiao, W., Q. Zhang, G. Habermacher, X. Yang, A.-y. Zhang, X. Cai, J. Hahn, et al. "U19/Eaf2 knockout causes lung adenocarcinoma, B-cell lymphoma, hepatocellular carcinoma and prostatic intraepithelial neoplasia." Oncogene 27, no. 11 (September 17, 2007): 1536–44. http://dx.doi.org/10.1038/sj.onc.1210786.

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Cecilie Alfsen, G., Klaus Beiske, Helge Bell, and Per F. Marton. "Low-grade intestinal lymphoma of intraepithelial T lymphocyties with concomitant enteropathy-associated T cell lymphoma: Case report suggesting a possible histogenetic relationship." Human Pathology 20, no. 9 (September 1989): 909–13. http://dx.doi.org/10.1016/0046-8177(89)90105-6.

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31

Aoki, Yasuhiro, Tomohisa Sujino, Kaoru Takabayashi, Makoto Mutakuchi, Katsura Emoto, Naoki Hosoe, Haruhiko Ogata, and Takanori Kanai. "Various Endoscopic Features in Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma." Case Reports in Gastroenterology 15, no. 1 (March 11, 2021): 312–22. http://dx.doi.org/10.1159/000513902.

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A 77-year-old female who had an acute severe abdominal pain was taken to the emergency room in the previous hospital. CT scans showed jejunum and ileum wall thickening and fatty deposits around the small intestinal tract, and gastrointestinal perforation could not be ruled out. By using single anal and oral balloon endoscopy, we observed mild edema with petechial erythema, shallow erosions with edematous mucosa and ulcers with surrounded disrupted villous structures at the jejunum and ileum. Histological analysis revealed atypical lymphocytes infiltrating the small intestinal mucosa demonstrating intraepithelial lymphocytosis. Immunohistochemical staining revealed that CD3, CD7, and CD56 staining was positive, and CD4, CD5, and CD8 staining was negative in infiltrated lymphocytes. We made the diagnosis of monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) with the combination of HE staining and IHC. PET-CT showed abnormal uptake in irregular wall thickening of the small intestine, lymph nodes, ribs, spine and pelvic bone. She was treated with chemotherapy (etoposide, prednisolone, oncovin, cyclophosphamide, hydroxydaunorubicin) and is still alive 1 year after the diagnosis. We reported the various endoscopic findings in the same MEITL patient by using single balloon endoscopy. We also summarized endoscopic characteristics of MEITL patients.

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Abdullah, Shahed Azzam Ahmed, Patricia Goa, Elisabeth Vandenberghe, and Richard Flavin. "Update on the Pathogenesis of Enteropathy-Associated T-Cell Lymphoma." Diagnostics 13, no. 16 (August 9, 2023): 2629. http://dx.doi.org/10.3390/diagnostics13162629.

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EATL is an aggressive T-cell non-Hodgkin lymphoma with poor prognosis and is largely localized to the small intestine. EATL is closely associated with coeliac disease (CD) and is seen mostly in patients originating from Northern Europe. Various factors are associated with an increased risk of developing EATL, such as viral infection, advanced age, being male, and the presence of the HLA-DQ2 haplotype. Clonal rearrangements in the TCR-β and γ genes have been reported in all EATL morphological variants with distinctive immunophenotypic characteristics. Although EATL can occur de novo, individuals with RCDII are at a higher risk of developing EATL. The cells of origin of EATL has been postulated to be normal small intestinal intraepithelial T-lymphocytes (IELs), and more recent evidence suggests a link between innate precursor IELs and EATL derived from refractory coeliac disease type II (RCDII). The immune microenvironment of mucosal cells within the small intestine enhances the process of neoplastic transformation of IELs into EATL. Cytokines such as IL-15 can activate and crucially deregulate the JAK-STAT signaling pathway by binding to receptors on the surface of IELs. Furthermore, mutations in the JAK/STAT pathway have been associated with RCDII-derived EATL.

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Rauch, Dan, Shimon Gross, John Harding, Stefan Niewiesk, Michael Lairmore, David Piwnica-Worms, and Lee Ratner. "Imaging spontaneous tumorigenesis: inflammation precedes development of peripheral NK tumors." Blood 113, no. 7 (February 12, 2009): 1493–500. http://dx.doi.org/10.1182/blood-2008-07-166462.

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AbstractEarly events in tumor development are spontaneous, microscopic, and affected by the microenvironment. We developed a mouse model of spontaneous lymphoma in which malignant transformation is coupled with light emission that can be detected noninvasively using bioluminescent imaging. The human T-cell leukemia virus (HTLV) type 1 transcriptional transactivator Tax is an oncogene sufficient to produce lymphoma in transgenic animal models. Using the granzyme B promoter to restrict Tax expression to the mature natural killer (NK)/T-cell compartment, we have reproduced many elements of HTLV-associated adult T-cell leukemia/lymphoma. Tax activates signaling cascades associated with transformation, inflammation, and tumorigenesis. Here, we report that Tax-mediated activation of luciferase in long terminal repeat-luciferase (LTR-LUC) mice serves as a reporter for imaging these processes in vivo. Using bioluminescent imaging (BLI), we discovered that microscopic intraepithelial lesions precede the onset of peripheral subcutaneous tumors, tumorigenesis progresses through early reversible stages, and Tax is sufficient for inducing tumors. Based on these findings, we propose that Tax expression in activated lymphocytes initiates a cascade of events that leads to NK/T cell recruitment, activation, and transformation. The use of BLI expands our ability to interrogate the role of Tax in tumorigenesis in vivo and has made the association of inflammation with tumor initiation amenable for study.

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Nijeboer, P., G. Malamut, C. J. Mulder, N. Cerf-Bensussan, D. Sibon, G. Bouma, C. Cellier, O. Hermine, and O. Visser. "Enteropathy-Associated T-Cell Lymphoma: Improving Treatment Strategies." Digestive Diseases 33, no. 2 (2015): 231–35. http://dx.doi.org/10.1159/000369542.

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Enteropathy-associated T-cell lymphoma (EATL) is a rare and usually rapidly fatal intestinal T-cell non-Hodgkin lymphoma. It arises from intraepithelial lymphocytes and has a high association with coeliac disease. The high mortality of EATL is associated not only with the very aggressive and often chemotherapy-refractory nature of the lymphoma. The poor condition of patients due to prolonged and severe malnutrition compromises the ability to deliver chemotherapy. There are no standardized treatment protocols, and the optimal therapy for EATL remains unclear. The primary step of treatment consists of local debulking, preferably as early as possible after EATL diagnosis. Morbidity and mortality seem to rise with advanced stages of disease due to tumour size progression, worse nutritional status and a higher risk of emergency surgery due to perforation. Standard induction therapy for EATL is anthracycline-based chemotherapy, preferably resumed between 2 and 5 weeks after surgery (depending on clinical condition). Intensification of therapy using high-dose chemotherapy followed by consolidation with BEAM and autologous stem cell transplantation is associated with better outcome. Notably, this treatment strategy has only been applied in patients eligible for this aggressive regimen which might reflect selection bias. Unfortunately, prognosis of EATL remains poor; 5-year survival varies from 8 to 60% depending on the eligibility to receive additional steps of therapy. New treatment strategies are urgently needed for a better prognosis of this lethal complication of coeliac disease. Brentuximab vedotin (anti-CD30) might be promising when added to conventional chemotherapy and is suggested as upfront treatment in EATL.

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Daum, Severin, Michael Hummel, Dajana Weiss, Michael Peters, Bertram Wiedenmann, Frank Schäper, Harald Stein, Ernst-Otto Riecken, and Hans-Dieter Foss. "Refractory Sprue Syndrome with Clonal Intraepithelial Lymphocytes Evolving into Overt Enteropathy-Type Intestinal T-Cell Lymphoma." Digestion 62, no. 1 (2000): 60–65. http://dx.doi.org/10.1159/000007779.

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Malamut, Georgia, and Christophe Cellier. "Refractory Celiac Disease: Epidemiology and Clinical Manifestations." Digestive Diseases 33, no. 2 (2015): 221–26. http://dx.doi.org/10.1159/000369519.

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A small subset of celiac disease (CD) patients becomes refractory to a gluten-free diet with persistent malabsorption and intestinal villous atrophy. This is a rare (probably less than 2% of adult CD patients), but serious disorder, with a high risk of progression to an overt T-cell lymphoma. Diagnosis of this condition defined as refractory CD (RCD) is made after exclusion of other small bowel diseases with villous atrophy. RCD has been subdivided into two subgroups according to the normal (RCDI) or abnormal phenotype of intraepithelial lymphocytes (RCDII). Whereas RCDI is hardly distinguishable from active noncompliant CD, RCDII has a severe clinical presentation and a very poor prognosis. We precisely describe below the different types of RCD and propose diagnostic and therapeutic guidelines for its clinical management.

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Cellier, Christophe, and Nadine Cerf-Bensussan. "Treatment of Clonal Refractory Celiac Disease or Cryptic Intraepithelial Lymphoma: A Long Road From Bench to Bedside." Clinical Gastroenterology and Hepatology 4, no. 11 (November 2006): 1320–21. http://dx.doi.org/10.1016/j.cgh.2006.09.011.

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38

Sibon, David, Sherine Khater, Julie Bruneau, Chantal Brouzes, Ludovic Lhermitte, Thierry Jo Molina, Guillaume Cartron, et al. "The Eatl-001 Trial: Results of a Phase 2 Study of Brentuximab Vedotin and CHP Followed By Consolidation with High-Dose Therapy - Autologous Stem-Cell Transplantation (HDT-ASCT) in the Frontline Treatment of Patients with Enteropathy-Associated T-Cell Lymphoma." Blood 138, Supplement 1 (November 5, 2021): 136. http://dx.doi.org/10.1182/blood-2021-153709.

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Abstract Background Enteropathy-associated T-cell lymphoma (EATL), previously designated type 1 EATL, is a neoplasm of intraepithelial T cells that occurs in individuals with celiac disease (CD). It is a rare lymphoma, accounting for approximately 3% of all peripheral T-cell lymphomas (PTCLs). EATL may be preceded by refractory CD (RCD), defined as persistent or recurrent symptoms and signs of malabsorption with villous atrophy despite a strict gluten-free diet for more than 12 months. Currently, RCD is categorized into 2 types, based on immunophenotypic and molecular criteria. In RCD-II, intraepithelial lymphocytes (IELs) have an aberrant phenotype and a clonal TCR gene rearrangement. RCD-II is considered a low-grade lymphoma of intraepithelial T cells, with a high risk of transformation into EATL. CD or RCD may be diagnosed prior to or concomitant with EATL. EATL has a poor prognosis due to perforation or obstruction of the bowel, sepsis, malnutrition and treatment resistance, with 2-year OS of 20% (de Baaij, CCR 2015). An EATL prognostic index (EPI) has been developed, that can distinguish 3 risk groups (de Baaij, CCR 2015). Optimal treatment of EATL is an unmet need, and novel therapeutic approaches are required. Most EATLs are CD30+ and could be targeted by brentuximab vedotin (BV). Based on the encouraging activity and manageable safety profile of BV and CHP (cyclophosphamide, doxorubicin and prednisone) combination in CD30+ PTCLs, the EATL-001 phase 2 trial was initiated to assess the efficacy and safety of BV-CHP followed by HDT-ASCT for the frontline treatment of patients (pts) with EATL (ClinicalTrials.gov No. NCT03217643). Here we report the first results of the EATL-001 trial. Methods EATL-001 is an Investigator Initiated-Sponsored Research phase 2 study, on behalf of the CELAC (French NCI-labeled network of Centers of Expertise for Lymphomas Associated with Celiac disease). Key inclusion criteria were as follows: Newly diagnosed CD30+ (≥10% of neoplastic cells by central review) EATL (WHO 2016 criteria), 18-65 years, PS 0-3. Response was assessed according to the Lugano classification. Pts were scheduled to receive 4 cycles of BV+CHP as induction. Responding pts received 2 cycles of Etoposide (200 mg/m2) + Methotrexate (3 g/m2) followed by HDT-ASCT (BEAM conditioning regimen). The primary endpoint was 2-year PFS per investigator. Underlying CD/RCD diagnosis was based on uninvolved duodenal histology (including CMF and TCR gene rearrangement analysis of IEL), serology and HLA typing. Results A total of 14 pts were included between February 2018 and February 2021. The median age was 54 years (range, 34-65) and 64% were male. 11 pts (79%) had initial surgery for bowel obstruction (n=6) or jejunal perforation (n=5). All pts had CD, diagnosed prior to (n=4) or concomitant with (n=10) EATL. 9 pts (64%) had RCD-II. CD30 expression ranged from 10% to 100%, nine cases having 100% positivity. EPI was high-risk in 4 pts (29%), intermediate-risk in 6 pts (43%), and low-risk in 4 pts (29%). Preliminary results by investigator assessment show an overall response rate following completion of the induction of 79% (11/14) with 64% (9/14) achieving a complete response. 3 pts had primary progressive disease (all had high-risk EPI), of which 2 died of the lymphoma. The 11 responding pts, still in response before intensification, underwent HDT-ASCT. 2 pts died of septic shock during HDT-ASCT. With a median follow-up of 2.1 years, there was no relapse and the 2-year PFS and OS for all pts were 63% and 68%, respectively. The incidence of AEs was consistent with the known safety profiles of BV-CHP regimen. Conclusions EATL-001 is the first prospective phase 2 study dedicated to EATL. BV-CHP was well tolerated and induced high response rates, allowing the majority of patients to be transplanted. This novel therapeutic approach shows promising efficacy compared to historical controls. Disclosures Sibon: Takeda: Consultancy; Roche: Consultancy; Janssen: Consultancy; Abbvie: Consultancy; iQone: Consultancy. Cartron: Roche, Celgene-BMS: Consultancy; Danofi, Gilead, Novartis, Jansen, Roche, Celgene-BMS, Abbvie, Takeda: Honoraria. Morschhauser: Incyte: Membership on an entity's Board of Directors or advisory committees; Celgene: Membership on an entity's Board of Directors or advisory committees; Gilead: Consultancy, Membership on an entity's Board of Directors or advisory committees; Chugai: Honoraria; AstraZenenca: Membership on an entity's Board of Directors or advisory committees; F. Hoffmann-La Roche Ltd: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees; AbbVie: Consultancy, Membership on an entity's Board of Directors or advisory committees; Novartis: Consultancy, Membership on an entity's Board of Directors or advisory committees; Genentech, Inc.: Consultancy; Janssen: Honoraria; Roche: Consultancy, Speakers Bureau; Epizyme: Consultancy, Membership on an entity's Board of Directors or advisory committees; BMS: Consultancy, Membership on an entity's Board of Directors or advisory committees; Genmab: Membership on an entity's Board of Directors or advisory committees; Servier: Consultancy. Hermine: Takeda: Consultancy. OffLabel Disclosure: Brentuximab vedotin is not approved in Europe for EATL.

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Grafanaki, Katerina, Spyridon Lygeros, Alexandros Spyridonidis, and Maria Liga. "Tongue graft-versus-host disease: remission with ruxolitinib." BMJ Case Reports 15, no. 5 (May 2022): e247888. http://dx.doi.org/10.1136/bcr-2021-247888.

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Graft-versus-host disease (GvHD) is a potentially life-threatening and commonly encountered event of allogeneic haematopoietic stem cell transplantation. Here, we present a young adult male with primary refractory Hodgkin’s lymphoma who received a transplant and developed cutaneous GvHD after donor lymphocyte infusion, which was managed with cyclosporine and steroids. However, while the patient was under immunosuppressive treatment, diffuse confluent whitish patches on the patient’s tongue were observed. A biopsy of the tongue lesions revealed lichenoid, hyperkeratotic tissue changes and intraepithelial T-cell infiltration consistent with chronic GvHD. He was treated with mycophenolate mofetil for 6 months with minimal improvement. Janus-associated kinase inhibitor (ruxolitinib) treatment was commenced, with complete resolution of the tongue lesions and treatment discontinuation 5 months later. Currently, 5 years after allogeneic transplantation, he is in remission and does not need immunosuppressive therapy.

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Radersma, Marijn, Laura R. de Baaij, Nathalie J. Hijmering, Gert Ossenkoppele, Chris JLM Meijer, Chris JJ Mulder, and Saskia AGM Cillessen. "hsTRAIL/Apo2L Induces Apoptosis in Enteropathy-Associated T-Cell Lymphoma." Blood 118, no. 21 (November 18, 2011): 1665. http://dx.doi.org/10.1182/blood.v118.21.1665.1665.

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Abstract Abstract 1665 EATL is an intestinal tumor of aberrant intraepithelial T-lymphocytes (IELs) and may be preceded by refractory celiac disease type II (RCD II). Current therapies include surgery, chemotherapy and autologous and/or allogeneic stem cell transplantation. Despite these therapies, the overall outcome of EATL is very poor with 1- and 5-year survival rates in the range of 31–39% and 8–20%, respectively. Therefore, new therapeutic options are needed. Human soluble tumor necrosis factor-related apoptosis-inducing ligand, hsTRAIL/Apo2L, a member of the TNF family, has proven to selectively kill tumor cells via an alternative, death-receptor mediated apoptosis pathway. In this present study we evaluated if hsTRAIL/Apo2L induces apoptosis in both isolated lymphoma cells of EATL biopsies and isolated cells of RCD II biopsies. hsTRAIL/Apo2L induced apoptosis in isolated EATL lymphoma cells. RCD II cells were less sensitive to hsTRAIL/Apo2L compared to EATL cells. hsTRAIL/Apo2L induced apoptosis in EATL cells was caspase-9 dependent, but unexpectedly active caspase-8 involvement could not be detected. RT-MLPA analysis on EATL samples confirmed this observation by showing increased levels of c-Flip in EATL cells, which suggests a blockage in the extrinsic apoptosis pathway. Both EATL and RCDII cells showed expression of TRAIL receptors R1 and R2 and almost no expression of R3 and R4. In conclusion, our study showed that hsTRAIL/Apo2L induces apoptosis in EATL cells through the intrinsic apoptosis pathway. Moreover, we showed that TRAIL receptor R1 and R2 are expressed in EATL cells. Based on these results, hsTRAIL/Apo2L may be a new therapeutic option for EATL patients. Disclosures: No relevant conflicts of interest to declare.

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Tóth, Gábor, Gábor László Sándor, Andrea Gyenes, Jeannette Tóth, Berthold Seitz, Zoltán Zsolt Nagy, and Nóra Szentmáry. "Szemfelszíni laphám-neoplasia." Orvosi Hetilap 158, no. 51 (December 2017): 2011–22. http://dx.doi.org/10.1556/650.2017.30939.

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Abstract: To summarize actual knowledge on epidemiology, etiology, pathology, clinical apparence and treatment of ocular surface squamous neoplasias. We summarize up-to-date literature on conjunctival intraepithelial neoplasia and invasive squamous cell carcinoma and present some own cases. Ocular surface squamous neoplasia is the most common malignant ocular surface tumor and the third most common ocular malignancy following malignant melanoma and lymphoma. In spite of its low malignant potential, in advanced stages it may reduce visual acuity significantly or even the eye globe has to be removed. In case of metastasis it may also be life-threatening. As local recurrences of ocular surface squamous neoplasias may occur, knowledge of intra- and postoperative adjuvant treatment options are indispensable and regular control examinations are necessary. Identification and adequate treatment of ocular surface squamous neoplasias are necessary in order to avoid its progression and to prevent recurrences. Orv Hetil. 2017; 158(51): 2011–2022.

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Sibon, David, Georgia Malamut, Virginie Verkarre, Coralie Derrieux, Isabelle Radford, Bertrand Meresse, Elizabeth Macintyre, et al. "Enteropathy-Associated T-Cell Lymphoma Type I, But Not Refractory Celiac Disease, Strongly Expresses CD30 and Might Benefit From Brentuximab Vedotin." Blood 122, no. 21 (November 15, 2013): 4252. http://dx.doi.org/10.1182/blood.v122.21.4252.4252.

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Abstract Introduction Enteropathy-associated T-cell lymphoma (EATL) is an intestinal tumor of intraepithelial T lymphocytes. The 2008 WHO classification distinguishes two types of EATL: the first type of EATL (type I), the most frequent (80-90%) is strongly associated with celiac disease (CD) and the HLA-DQ2/DQ8 haplotypes. The tumor cells are CD3+CD8-/+CD4-CD56- and contain cytotoxic granules. In almost all cases, a varying proportion of the tumor cells express CD30. The second type of EATL (type II), the monomorphic form, has a distinct immunophenotype (CD3+CD4-CD8+CD56+). In two types, TCRgamma genes are often clonally rearranged. EATL may be preceded by refractory celiac disease (RCD), corresponding to CD refractory to gluten free diet (GFD). RCD is divided in two types based on the absence (type I) or presence (type II) of abnormal intraepithelial lymphocytes (IEL) showing down-regulation of CD8 and often TCRgamma genes rearrangement. RCD II is now considered as a small cell intraepithelial T-cell lymphoma that could be an intermediate stage between CD and EATL. The aim of the present study was to establish the pattern of CD30 expression in EATL. This could have therapeutic implications with the use of anti-CD30 monoclonal antibody like brentuximab vedotin (BV). Methods Consecutive adult patients (pts) diagnosed with EATL between 2007 and 2013 in two university hospitals in Paris (Necker University Hospital and Georges Pompidou European Hospital) were eligible for this study. Diagnosis was confirmed after histopathologic and immunohistochemical review. For the purpose of the present study, two expert hematopathologists (V.V. and N.B.) reviewed all EATL and extended the phenotypic analysis to reclassify them according to the 2008 WHO classification. A panel of antibodies directed against CD20, CD3, CD4, CD5, CD8, CD56, granzyme B and ALK1 was used. CD30 staining was performed with Monoclonal Mouse Anti-Human CD30, Clone Ber-H2 (Dako). Consecutive RCDI and RCDII cases with complete phenotype and clonality analyses diagnosed in the same period were used as control. Diagnosis of CD was based on HLA-DQ2/8 typing, detection of celiac specific antibodies and of villous atrophy with increased counts of IEL on normal diet. Pts were further classified in RCDI or II depending on their clinical and histological response to a GFD and the presence of abnormal IEL. Results Twenty five adults were diagnosed with EATL on consensus review (median age 53 years [range 34-76], M/F ratio 12/13). Twenty five RCDI pts (median age 51 years [range 16-75], M/F ratio 6/19) and 20 RCDII pts (median age 62 years [range 29-81], M/F ratio 7/13) were used as control. A clinical history of CD was found in 17/20 (85%) evaluable EATL pts. Histological features of CD/RCD were seen in all cases (20/20) of EATL in which the mucosa adjacent to the tumor could be investigated (half of these were RCDII). Primary sites of EATL were small intestine (20/25), mesenteric lymph nodes (3/25), peritoneal nodules (1/25) and spleen (1/25). Phenotypic analysis showed that EATL cases were all WHO type I (25/25). ALK1 was constantly negative. IEL were CD3+ in all 70 cases. CD8 was normally expressed in all RCDI IEL and downregulated in all RCDII IEL and 35% of EATL. In all cases of EATL (25/25), CD30 was strongly expressed by all large tumor cells. In RCDI and II, CD30 was negative in most cases (∼90%), and was rarely expressed by dispersed atypical lymphocytes (IEL or in lamina propria) in some cases. TCRgamma genes were clonally rearranged in 11/14 (79%) EATL, 3/25 (12%) RCDI and 18/20 (90%) RCDII. Based on these results, we initiated in 2012 a pilot study combining BV with chemotherapy followed by autologous stem-cell transplantation (ASCT) as frontline treatment of EATL. Five pts have currently been treated. The associated chemotherapy regimen was IVE/MTX (Sieniawski M, Blood 2010) for the first two pts. After presentation at ASH 2012 Annual Meeting of preliminary results of a phase 1 study combining BV with CHP regimen as frontline treatment of systemic ALCL and other CD30-positive mature T–cell and NK–cell lymphomas (Fanale MA, Abstract #60), we replaced IVE/MTX by CHP regimen, and treated 3 other pts. The treatment was well tolerated, and the 5 pts reached CR and underwent ASCT. Conclusion CD30 is strongly expressed in EATL type I. Promising results of the combination of BV with CHP led us to plan a phase 2 study of BV and CHP followed by ASCT as frontline treatment of EATL. Disclosures: Off Label Use: Brentuximab vedotin was used in enteropathy-associated T-cell lymphoma (EATL).

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Qiu, W., H. Khokhar, T. A. Dewenter, and R. Jetly. "Challenge in Early Diagnosis of Rare Lymphomas – A Case of Monomorphic Epitheliotropic Intestinal T-cell Lymphoma Masquerading as Enteritis." American Journal of Clinical Pathology 156, Supplement_1 (October 1, 2021): S91—S92. http://dx.doi.org/10.1093/ajcp/aqab191.195.

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Abstract Introduction/Objective Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is an extranodal lymphoma arising from intestinal intraepithelial T-lymphocytes. Diagnosis is challenging obscured by non-specific gastrointestinal (GI) symptoms and radiographic findings, with potential delay in implementing therapy, as was encountered in a 40- year-old man of African-American descent with cachexia. Methods/Case Report The patient presented with a two month history of nausea, vomiting, diarrhea and 10-15 pounds weight loss. Computed tomography revealed wall thickening of multiple large- and small-bowel loops. The clinical differential diagnoses included infectious enteritis and inflammatory bowel disease. Since symptoms improved during the hospital stay, he was discharged with future outpatient endoscopy planned but returned a month later with abdominal swelling and pain. On repeat imaging, he had ascites and small-bowel mechanical obstruction versus ileus. Straw-colored ascites, dilated friable segments of small-bowel, matted mesenteric lymph nodes, and adhesions were found on exploratory laparotomy. Small-bowel segments and appendix were resected. A prolonged hospital course ensued post-operatively with death due to multiple complications. Results (if a Case Study enter NA) The intestinal wall was grossly thickened with no discrete masses and microscopically had extensive infiltrates of small- to intermediate-sized, mature, minimally pleomorphic, CD3+/CD4- /CD5-/CD7+/CD8+/CD10-/CD20-/CD30-/CD56+/EBER-ISH- T-lymphocytes, focally infiltrating into the epithelium. Ascitic fluid cytology had small lymphocytes, CD2+/CD3+/CD4-/CD5-/CD10-/CD7+/CD8+/CD25-/CD30-/CD56+ /TCRαβ+/TCRγδ- by flow cytometry immunophenotyping. A diagnosis of MEITL was rendered based on these findings. Conclusion MEITL is rare, aggressive, unassociated with celiac disease and usually seen in Asian and Hispanic populations. It presents with non-specific GI symptoms of abdominal pain, perforation, diarrhea, weight loss and intestinal obstruction underscoring the need for a high index of suspicion. Upper GI endoscopy has a limited role since involvement of small-bowel, specifically jejunum, is common. The evolving capsule endoscopy techniques may help in an early diagnosis. Due to limited literature, optimal treatment is unclear and various strategies using chemotherapy, surgery and stem-cell transplant have been tried with variable outcomes.

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Zweig, J. I. "Eradication of prostatic high grade intraepithelial neoplasia: Hypothesis and clinical proof." Journal of Clinical Oncology 25, no. 18_suppl (June 20, 2007): 1534. http://dx.doi.org/10.1200/jco.2007.25.18_suppl.1534.

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1534 Background: Recently normal prostate tissue has been found to contain a high concentration of retinoid X receptors (RXRs), they are found in the highest concentrations in ectodermal tissue. Loss of these receptors is associated with prostatic intraepithelial neoplasia (PIN), and can be present in patients with a normal PSA. There are 115,000 cases of high grade PIN discovered yearly in the United States.The drug bexarotene used to treat cutaneous T cell lymphoma (CTCL). The compound is not a true retinoid in structure, perusal of the structure reveals two aromatic rings one of which contains a carboxy group which would make one anticipate strong hydrogen bonding when it undergoes binding to the RXRs as if it were a retinoid, resulting in “up regulation” of the tissue with increase in differentiation to normal tissue. Based upon this it was felt that it would be fruitful to try bexarotene in High Grade PIN. Method: The diagnosis was based upon 12 biopsies which were done in conjunction with ultrasound localization of the biopsy site, with careful recording of there anatomical position. The patient was started on bexarotene 225 mg per day. The drug was given as a single dose once daily; this dose is 40% of the dose normally used to treat CTCL. The drug was administered for 30 doses During the period of therapy the patient was on Atorvastin T4 total was within the normal range. Results: Four weeks following the last dose of bexarotene the prostate was rebiopsied with 12 biopsies taken. Six of the biopsies were taken from the putative area, the rest in an enlarging target like pattern. The repeat biopsy was negative for the presence of PIN. 14 months later 12 more biopsies were obtained using the same technique, these repeat biopsies were also found to be negative; ie. 24 negative biopsies over 14 months. It is unlikely that the previously involved area was missed. Conclusion: If a mechanism of action were to be formulated, the induction of and increase in RXRs in normal prostate cells would be reasonable. Cells that undergo the formation of PIN, strong hydrogen bonding and inability of those cells to produce more receptors could result in apoptosis. Undoubtedly further study is required to evaluate the effect of this drug in high grade PIN. No significant financial relationships to disclose.

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Ondrejka, Sarah Lynn, Andrea B. Moffitt, Eric Tse, Eric D. Hsi, John R. Goodlad, Rex au-Yeung, Yok-Lam Kwong, et al. "Whole Exome Sequencing of Type 1 and Type 2 Enteropathy-Associated T Cell Lymphoma Reveals Genetic Basis of Eatl Oncogenesis." Blood 126, no. 23 (December 3, 2015): 575. http://dx.doi.org/10.1182/blood.v126.23.575.575.

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Abstract Introduction Enteropathy-associated T cell lymphoma (EATL) is an intestinal tumor of the intraepithelial T lymphocytes, with a median survival time of less than 1 year. It is a rare disease in general and has two main subtypes described. Type 1 EATL is a complication in patients with celiac disease, a chronic gluten-sensitive enteropathy. Type 2 EATL, characterized by smaller monomorphic lymphocytes, typically occurs sporadically in patients without celiac disease. Very little is known about the genetic mutations and gene expression signatures that define this disease, or the extent to which the two types of EATL are genetically distinct. It has been suggested that the two types of EATLs should be reclassified as separate diseases in future WHO categories. Methods In this study, we performed whole exome sequencing to 100-fold depth of 41 EATL tumors including 23 type 1 cases and 18 type 2 cases. Both alpha-beta (65%) and gamma-delta (35%) T cell receptor rearrangements were seen among these cases. Paired normal DNA was sequenced in most (N=30) cases. We defined somatic mutations, copy number alterations, and HLA genotypes in these cases from sequencing data. Additionally, we generated RNA sequencing data on the same EATL tumors. Corresponding clinical and outcome data was collected on the same cohort. Results We found that both type 1 and type 2 EATLs had overlapping patterns of mutations and similar overall survival. The most commonly mutated genes were chromatin modifier genes (34%) including ATRX and ARID1B. We also identified recurrent somatic mutations in signal transduction genes, including JAK1 and BCL9L. TP53 mutations were also recurrent (12%). Copy number amplifications in 9q, 1q, and 8q occurred most frequently and were present in both subtypes. We further compared the mutational profiles to peripheral T cell lymphoma, angioimmunoblastic T cell lymphoma, cutaneous T cell lymphoma, natural killer/T cell lymphoma, diffuse large B cell lymphoma, and Burkitt lymphoma. These comparisons identify EATL as a genetically distinct disease with a very different pattern of mutations. RNAseq identified the gene expression patterns that are unique to EATL and also identified gene expression signatures that distinguish the two types of EATL. The DQ2 or DQ8 HLA genotype is present in the majority of type 1 cases (73%) while occurring infrequently in type 2 cases (27%). Conclusions Our study defines the genetic landscape of enteropathy associated T cell lymphoma and highlights the genetic and clinical overlap between the two types. While the two types have differences in mutations and gene expression patterns, they have more in common with each other compared to other lymphoma types. Our data may inform future decisions regarding the potential separation of the two EATL types as distinct entities. Disclosures No relevant conflicts of interest to declare.

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Daum, S. "Frequency of clonal intraepithelial T lymphocyte proliferations in enteropathy-type intestinal T cell lymphoma, coeliac disease, and refractory sprue." Gut 49, no. 6 (December 1, 2001): 804–12. http://dx.doi.org/10.1136/gut.49.6.804.

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Chuang, S.-S., Y.-L. Liao, H. Liu, S.-H. Lin, P.-P. Hsieh, W.-T. Huang, H.-K. Chen, and P. G. Isaacson. "The phenotype of intraepithelial lymphocytes in Taiwanese enteropathy-associated T-cell lymphoma is distinct from that of the West." Histopathology 53, no. 2 (August 2008): 234–36. http://dx.doi.org/10.1111/j.1365-2559.2008.03064.x.

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Spencer, J., T. T. MacDonald, T. C. Diss, J. A. Walker-Smith, P. J. Ciclitira, and P. G. Isaacson. "Changes in intraepithelial lymphocyte subpopulations in coeliac disease and enteropathy associated T cell lymphoma (malignant histiocytosis of the intestine)." Gut 30, no. 3 (March 1, 1989): 339–46. http://dx.doi.org/10.1136/gut.30.3.339.

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Verbeek, Wieke H. M., B. Mary E. von Blomberg, Petra E. T. Scholten, D. Joop Kuik, Chris J. J. Mulder, and Marco W. J. Schreurs. "The Presence of Small Intestinal Intraepithelial Gamma/Delta T-Lymphocytes Is Inversely Correlated With Lymphoma Development in Refractory Celiac Disease." American Journal of Gastroenterology 103, no. 12 (December 2008): 3152–58. http://dx.doi.org/10.1111/j.1572-0241.2008.02213.x.

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Kooy-Winkelaar, Yvonne M. C., Dagmar Bouwer, George M. C. Janssen, Allan Thompson, Martijn H. Brugman, Frederike Schmitz, Arnoud H. de Ru, et al. "CD4 T-cell cytokines synergize to induce proliferation of malignant and nonmalignant innate intraepithelial lymphocytes." Proceedings of the National Academy of Sciences 114, no. 6 (January 3, 2017): E980—E989. http://dx.doi.org/10.1073/pnas.1620036114.

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Refractory celiac disease type II (RCDII) is a severe complication of celiac disease (CD) characterized by the presence of an enlarged clonal population of innate intraepithelial lymphocytes (IELs) lacking classical B-, T-, and natural killer (NK)-cell lineage markers (Lin−IELs) in the duodenum. In ∼50% of patients with RCDII, these Lin−IELs develop into a lymphoma for which no effective treatment is available. Current evidence indicates that the survival and expansion of these malignant Lin−IELs is driven by epithelial cell-derived IL-15. Like CD, RCDII is strongly associated with HLA-DQ2, suggesting the involvement of HLA-DQ2–restricted gluten-specific CD4+ T cells. We now show that gluten-specific CD4+ T cells isolated from CD duodenal biopsy specimens produce cytokines able to trigger proliferation of malignant Lin−IEL lines as powerfully as IL-15. Furthermore, we identify TNF, IL-2, and IL-21 as CD4+ T-cell cytokines that synergistically mediate this effect. Like IL-15, these cytokines were found to increase the phosphorylation of STAT5 and Akt and transcription of antiapoptotic mediator bcl-xL. Several small-molecule inhibitors targeting the JAK/STAT pathway blocked proliferation elicited by IL-2 and IL-15, but only an inhibitor targeting the PI3K/Akt/mTOR pathway blocked proliferation induced by IL-15 as well as the CD4+ T-cell cytokines. Confirming and extending these findings, TNF, IL-2, and IL-21 also synergistically triggered the proliferation of freshly isolated Lin−IELs and CD3−CD56+ IELs (NK-IELs) from RCDII as well as non-RCDII duodenal biopsy specimens. These data provide evidence implicating CD4+ T-cell cytokines in the pathogenesis of RCDII. More broadly, they suggest that adaptive immune responses can contribute to innate IEL activation during mucosal inflammation.

Journal articles on the topic 'Intraepithelial lymphoma'

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