• Contrast-enhanced computed tomography
• Gastrointestinal tract
• Lymphoma, Extranodal NK-T-cell

• 2021A1515011795 the Natural Science Foundation of Guangdong Province
• 2023A1515010333 the Natural Science Foundation of Guangdong Province
• 2023A1515011292 the Natural Science Foundation of Guangdong Province

• Humans
• Intestinal Perforation
• Lymphoma, Extranodal NK-T-Cell
• Skin Neoplasms/pathology

• T cell lymphoma
• asparaginase/therapeutic use
• efficacy
• physiopathology
• retrospective analysis

• ALCL
• MEITL
• NK/T-cell lymphoma
• PTCL
• T-cell lymphoma

• PD-1/PD-L1 inhibitor
• asparaginase
• extranodal NK/T-cell lymphoma
• immunotherapy
• novel drug
• review
• treatment

• Epstein–Barr virus
• immunosuppression
• inflammatory bowel disease
• lymphoproliferative diseases
• viral colitis

• Humans
• Epstein-Barr Virus Infections
• Herpesvirus 4, Human
• Inflammatory Bowel Diseases/complications
• Inflammatory Bowel Diseases/drug therapy
• Inflammatory Bowel Diseases/diagnosis
• Colitis, Ulcerative/therapy
• Lymphomatoid Granulomatosis

• extranodal NK/T-cell lymphoma
• gastrointestinal
• nomogram
• prognosis
• survival

• CD8αα+ T-cells
• EATL
• ENKTL
• ITCL
• Indolent T-cell LPD
• MEITL
• NK cells
• NK-cell enteropathy
• NOS
• iCD3+ ILC
• iCD8α ILC
• lymphomatoid gastropathy

• OF-LCG18May-0028 National Medical Research Council
• CSSSP20nov-0019 National Medical Research Council

• EATL
• ITCL
• MEITL
• NKCE
• NKTCL
• indolent T-cell LPD

Background: Extranodal NK/T cell lymphoma is a rare non-Hodgkin lymphoma mainly involving the upper aerodigestive tract, even rarer is primary extranasal disease involving the intestine. We present a case of primary intestinal NK/T cell lymphoma with diagnostic challenge, which eventually developed into multiple intestinal perforations.

Case Presentation: A 35-year-old man presented with diarrhea and recurrent fever. Abdominal CT revealed multi-segmental intestinal wall thickening. Colonoscopy showed multiple irregular ulcers in colon. During the hospitalization, the patient developed intestinal perforation and an emergency surgery was performed. The resected specimen showed multiple perforations of the colon. The surgical samples underwent pathological analysis, and a diagnosis of extranodal NK/T cell lymphoma nasal type was confirmed. After recovering from surgery, the patient started receiving chemotherapy and PD-1 monoclonal antibody. Fortunately, he was discharged after significant improvement in his general condition. Eleven months follow-up was uneventful.

Conclusion: Early diagnosis of primary intestinal NK/T cell lymphoma is frequently difficult. Most patients were definitely diagnosed only after surgical resection following complications, resulting in a poor prognosis. Therefore, doctors should maintain high suspicion of this malignancy for early diagnosis at an early stage clinically.

• Intestinal neoplasm
• Nk/t cell lymphoma
• extranodal
• non-Hodgkin lymphoma
• perforation

• ATLL
• Colorectum
• Lymphocytic proctocolitis
• MEITL
• T/NK-cell lymphoma

• Adult
• Aged
• Aged, 80 and over
• Colorectal Neoplasms/pathology
• Endoscopy, Digestive System
• Enteropathy-Associated T-Cell Lymphoma/pathology
• Female
• Humans
• Lymphoma, Extranodal NK-T-Cell/pathology
• Male
• Middle Aged

The incidence of intestinal NK/T cell lymphoma (NKTCL) is extremely low, and the clinical symptoms are atypical, which makes it difficult to distinguish this disorder from Crohn's disease (CD), T lymphocyte proliferative disease, and other immune disorders. The misdiagnosis rate is high, and the patient's prognosis is poor.

In this case, the patient had repeated high fever, colonoscopy revealed multiple ulcers, and the initial diagnosis was CD. The patient’s condition did not improve after treatment with hormones and infliximab, and she eventually died. Positron emission tomographic-computed tomographic and B-ultrasound were performed in our hospital and showed that multiple lymph nodes were enlarged. Immunohistochemi-stry showed that CD3 and Epstein-Barr virus encoded RNA expression was positive. Colonoscopy, tissue biopsy, and histopathology showed intestinal focal mucosal infiltration of heterotypic lymphocytes with an abnormal immune phenotype. On the basis of the patient’s medical history, auxiliary examination, and pathological findings, digestive physicians and pathologists gave the diagnosis of NKTCL.

Clinicians need to improve their comprehensive knowledge of NKTCL, and combination of clinical symptoms, histological characteristics, as well as colonoscopy biopsies should be considered to improve the diagnosis and thereby reduce misdiagnosis.

• NK/T cell lymphoma
• Indolent T lymphocyte proliferative disease
• Crohn's disease
• Endoscopy
• Misdiagnosis
• Case report

• Adult
• Crohn Disease/diagnosis
• Diagnosis, Differential
• Enteropathy-Associated T-Cell Lymphoma
• Epstein-Barr Virus Infections/diagnosis
• Female
• Herpesvirus 4, Human
• Humans
• Intestines

• Case report
• Multidisciplinary team discussion
• Primary intestinal extranodal natural killer/T-cell lymphoma
• Rare disease

• Adult T-cell leukemia/lymphoma (ATLL)
• endoscopy
• gastroenteropathy
• gastrointestinal tract

• Clinicopathologic
• Intestinal neoplasm
• NK/T lymphoma
• Primary

• chronic active EBV infection
• intestinal T/NK cell lymphoma
• intra-epithelial lymphocytosis T cell receptor

• Chronic active EBV infection
• EBV+ mucocutaneous ulcer
• Extranodal NK/T-cell lymphoma
• Hydroa vacciniforme

To investigate the clinicopathological features, survival and prognostic factors of primary intestinal extranodal natural killer/T-cell lymphoma, nasal type (PI-ENKTCL).

Clinical and histological characteristics of PI-ENKTCL cases were retrospectively evaluated. Immunohistochemical phenotype and status of Epstein-Barr virus (EBV) and T-cell receptor (TCR) gene rearrangement were examined. The overall survival and prognostic parameters were also analyzed.

Fifty-five (2.7%) cases with PI-ENKTCL were identified out of 2017 archived ENKTCL cases, with a median age of 39 years and a male to female ratio of 2.1:1. The most common symptom was abdominal pain (90.9%), accompanied frequently with fever and less commonly with intestinal perforation or B symptoms. Small intestine (50.9%) was the most common site to be involved. 47.3% and 36.4% cases presented with stage I and II diseases, respectively. Histologically, most cases displayed characteristic morphologic changes of ENKTCL. Cytoplasmic CD3, TIA-1 and CD56 expression was found in 100%, 94.5% and 89.1% of cases, respectively. In situ hybridization detection for EBV demonstrated positive results in all cases. Monoclonal TCR gene rearrangement was found in 52.9% of tested cases. Chemotherapy with a DICE or L-asparaginase/peg-asparginase-containing regimen was most often employed. Both advanced tumor stage and B symptoms were independent inferior prognostic factors (p = 0.001 and p = 0.010). Noticeably, 6 cases demonstrated a CD4-positive phenotype. These cases featured a relatively older median age (58 years), predominance of small/medium-sized neoplastic cells, a higher rate of TCR rearrangement and slightly favorable outcome.

We reported by far the largest series of PI-ENKTCL, and demonstrated its heterogeneity, aggressive clinical behavior and unsatisfying response to the current therapeutic strategies. Those CD4-positive cases might represent a unique subtype of PI-ENKTCL or distinct entity. Further investigations are required for the better understanding and management of this unusual disease.

• Adolescent
• Adult
• Aged
• Female
• Humans
• Ileum/metabolism
• Ileum/pathology
• Immunohistochemistry
• In Situ Hybridization
• Intestinal Mucosa/metabolism
• Intestines/pathology
• Kaplan-Meier Estimate
• Lymphoma, T-Cell/metabolism
• Lymphoma, T-Cell/pathology
• Male
• Middle Aged
• Natural Killer T-Cells/metabolism
• Natural Killer T-Cells/pathology
• Polymerase Chain Reaction
• Receptors, Antigen, T-Cell/genetics
• Receptors, Antigen, T-Cell/metabolism
• Retrospective Studies
• Young Adult

了解非上呼吸消化道原发NK/T细胞淋巴瘤（NUAT-NKTCL）患者的临床特征及生存情况。

收集44例NUAT-NKTCL患者的临床资料，对其临床特征、疗效及生存进行相关性分析。

44例患者中男31例，女13例，中位年龄39（15～82）岁，Ⅲ/Ⅳ期者27例（61.4%）。就诊时主要症状为皮肤肿物（21/44）、腹痛（11/44）、睾丸肿大（3/44）及间断咳嗽（2/44）等。存在B症状者28例（63.6%），ECOG评分≥2分者12例（27.3%），IPI评分≥3分者18例（40.9%），LDH升高者占48.8%（21/43）。全组患者均接受化疗为主的治疗，中位随访13.5（0.3～121.0）个月，共有32例患者死亡，中位生存时间为16个月，1年总生存率为54.1%。按照一线治疗方案分组：CHOP或CHOPE方案治疗组26例，完全缓解（CR）5例（19.2%），其中有19例患者接受左旋门冬酰胺酶（L-ASP）为主的挽救化疗，9例（47.7%）获CR，中位随访时间为11个月，中位生存时间为13个月；L-ASP为主的方案一线治疗组18例，CR 10例（55.6%），中位随访时间为15个月，中位生存时间为16个月。后者较前者CR率提高（P= 0.015），但两组患者总生存时间差异无统计学意义（P=0.774）。

NUAT-NKTCL侵袭性强，患者起病时分期晚。与传统方案比较采用L-ASP为主的方案可提高NUAT-NKTCL患者的疗效，但其长期生存与上呼吸消化道原发患者相比仍不理想，需要探索更为有效的治疗策略。

Extranodal natural killer/T-cell lymphoma, nasal type, is a rare type of non-Hodgkin cell lymphoma endemic to East Asia and parts of Central and South America. In most cases, it is driven by Epstein-Barr virus infections, with a broad range of morphologic appearances, frequent necrosis, and angioinvasion. It is designated as NK/T reflecting uncertainty in its cellular origins. These tumors usually arise in the nasal region, typically presenting with symptoms of nasal obstruction, epistaxis, and/or a destructive mass involving the nose, sinuses, or palate. The treatment of patients with extranodal NK/T-cell lymphoma, nasal type, is largely determined by the extent of disease. Localized disease is usually treated with radiation and chemotherapy. The disseminated disease requires combination chemotherapy. This report describes the case of a 30-year-old Caucasian female presenting with a left breast mass of two months duration. Excisional biopsy was done, and the pathological exam confirmed the diagnosis of extranodal NK/T-cell lymphoma, nasal type. Our patient received a systemic combination chemotherapy with steroid (dexamethasone), methotrexate, ifosfamide, L-asparaginase, and etoposide (SMILE) regimen, resulting in a complete clinical and radiological remission. On the basis of our review of the literature, extranodal NK/T non-Hodgkin cell lymphoma, nasal type, presenting as a breast mass is very rare and very uncommon in the United States. Awareness of this occurrence may be valuable as this case may be a forerunner of additional similar cases developing in the future.

• breast ca
• epstein-barr virus (ebv)
• extra-nodal nk/t-cell lymphoma
• smile regimen

Gastrointestinal lymphomas represent up to 10% of gastrointestinal malignancies and about one third of non-Hodgkin lymphomas. The most prominent histologies are mucosa-associated lymphoid tissue lymphoma and diffuse large B-cell lymphoma. However, the gastrointestinal tract can be the site of rarer lymphoma subtypes as a primary or secondary localization. Due to their rarity and the multifaceted histology, an endoscopic classification has not been validated yet. This review aims to analyze the endoscopic presentation of rare gastrointestinal lymphomas from disease diagnosis to follow-up, according to the involved site and lymphoma subtype. Existing, new and emerging endoscopic technologies have been examined. In particular, we investigated the diagnostic, prognostic and follow-up endoscopic features of T-cell and natural killer lymphomas, lymphomatous polyposis and mantle cell lymphoma, follicular lymphoma, plasma cell related disease, gastrointestinal lymphomas in immunodeficiency and Hodgkin’s lymphoma of the gastrointestinal tract. Contrarily to more frequent gastrointestinal lymphomas, data about rare lymphomas are mostly extracted from case series and case reports. Due to the data paucity, a synergism between gastroenterologists and hematologists is required in order to better manage the disease. Indeed, clinical and prognostic features are different from nodal and extranodal or the bone marrow (in case of plasma cell disease) counterpart. Therefore, the approach should be based on the knowledge of the peculiar behavior and natural history of disease.

• Endoscopy
• Lymphoma
• Endosonography
• Stomach
• Intestine