Monomorphic epitheliotropic intestinal T-cell lymphoma with bone marrow involved: A case report

BACKGROUND Monomorphic epithelial intestinal T-cell lymphoma(MEITL)is a rare type of peripheral T-cell lymphoma.The clinical manifestations are diarrhea,abdominal pain,perforation and an abdominal mass.CASE SUMMARY We present a 52-year-old female patient who was diagnosed with MEITL.Further disease progression was observed after multiline chemotherapy.Eventually,the patient died of a severe infection.CONCLUSION MEITL is a rare intestinal primary T-cell lymphoma with aggressive behavior,a high risk of severe life-threatening complications,and a poor prognosis.

Intestinal T-cell lymphomas:A retrospective analysis of 68 cases in China

AIM: To investigate the clinical features, diagnosis, treatment and prognosis of intestinal T-cell lymphomas (ITCL) by retrospective analysis.

Monomorphic epitheliotropic intestinal T-cell lymphoma presenting as melena with long-term survival: A case report and review of literature

BACKGROUND Monomorphic epitheliotropic intestinal T-cell lymphoma(MEITL)is a rare primary intestinal T-cell lymphoma,previously known as enteropathy-associated T-cell lymphoma type II.MEITL is an aggressive T-cell lymphoma with a poor prognosis and high mortality rate.The known major complications of MEITL are intestinal perforation and obstruction.Here,we present a case of MEITL that was diagnosed following upper gastrointestinal bleeding from an ulcerative duodenal lesion,with recurrence-free survival for 5 years.CASE SUMMARY A 68-year-old female was admitted to our hospital with melena and mild anemia.An urgent esophagogastroduodenoscopy(EGD)revealed bleeding from an ulcerative lesion in the transverse part of the duodenum,for which hemostatic treatment was performed.MEITL was diagnosed following repeated biopsies of the lesion,and cyclophosphamide,doxorubicin,vincristine,and prednisone(CHOP)chemotherapy was administered.She achieved complete remission after eight full cycles of CHOP therapy.At the last follow-up examination,EGD revealed a scarred ulcer and 18Fluorodeoxyglucose(18FDG)positron emission tomography/computed tomography showed no abnormal FDG accumulation.The patient has been in complete remission for 68 mo after initial diagnosis.CONCLUSION To rule out MEITL,it is important to carefully perform histological examination when bleeding from a duodenal ulcer is observed.

Primary intestinal extranodal natural killer/T-cell lymphoma, nasal type: A case report

BACKGROUND Primary intestinal extranodal natural killer/T-cell lymphoma,nasal type(PIENKTCL)is a rare non-Hodgkin’s lymphoma(NHL)subtype,and its prognosis is extremely poor.Clinical characteristics of the disease are not obvious and easily misdiagnosed.In this case report,we describe a patient with PI-ENKTCL who presented with intermittent hematochezia.The advantages of positron emission tomography/computed tomography(PET-CT)as a useful diagnostic tool and the role of surgery as an important therapy are highlighted.CASE SUMMARY A 45-year-old man,hospitalized due to intermittent hematochezia,underwent gastroscopy,colonoscopy,biopsy and CT,but no cause was found.Hence,we carried out a multidisciplinary team(MDT)discussion on the causes and treatment of this patient,and it was decided to perform PET-CT imaging with a MDT discussion of the results.PET-CT demonstrated a diagnosis of lymphoma and it was decided to surgically resect the lesion,and a R0 resection was successfully performed.Postoperative pathology showed negative resection margins,and examination of the lesion confirmed the diagnosis of PI-ENKTCL.After surgery,the patient underwent a follow-up period of 6 mo and received 6 cycles of gemcitabine,oxaliplatin and L-asparaginase.No recurrence or metastasis occurred.CONCLUSION PI-ENKTCL is rare,and MDT discussion is required during diagnosis.PET-CT can be performed for imaging diagnosis.Treatment is based on surgical resection,and the best treatment regimen is determined according to postoperative pathological results to improve prognosis and to extend survival in patients.

Intestinal natural killer/T-cell lymphoma presenting as a pancreatic head space-occupying lesion:A case report

BACKGROUND Intestinal natural killer/T-cell lymphoma(NKTCL)is a rare and aggressive non-Hodgkin’s lymphoma,and its occurrence is closely related to Epstein-Barr virus infection.In addition,the clinical symptoms of NKTCL are not obvious,and the specific pathogenesis is still uncertain.While NKTCL may occur in any segment of the intestinal tract,its distinct location in the periampullary region,which leads clinicians to consider mimics of a pancreatic head mass,should also be addressed.Therefore,there remain huge challenges in the diagnosis and treatment of intestinal NKTCL.CASE SUMMARY In this case,we introduce a male who presented to the clinic with edema of both lower limbs,accompanied by diarrhea,and abdominal pain.Endoscopic ultrasound(EUS)showed well-defined homogeneous hypoechoic lesions with abundant blood flow signals and compression signs in the head of the pancreas.Under the guidance of EUS-fine needle biopsy(FNB)with 19 gauge or 22 gauge needles,combined with multicolor flow cytometry immunophenotyping(MFCI)helped us diagnose NKTCL.During treatments,the patient was prescribed the steroid(dexamethasone),methotrexate,ifosfamide,L-asparaginase,and etoposide chemotherapy regimen.Unfortunately,he died of leukopenia and severe septic shock in a local hospital.CONCLUSION Clinicians should enhance their understanding of NKTCL.Some key factors,including EUS characteristics,the right choice of FNB needle,and combination with MFCI,are crucial for improving the diagnostic rate and reducing the misdiagnosis rate.

Surgical management of monomorphic epitheliotropic intestinal Tcell lymphoma followed by chemotherapy and stem-cell transplant:A case report and review of the literature

BACKGROUND Monomorphic epitheliotropic intestinal T-cell lymphoma(MEITL)is a rare and rapidly progressive intestinal T-cell non-Hodgkin lymphoma associated with a very poor prognosis and a median survival of 7 mo.Advances in the identification of MEITL over the last two decades have led to its recognition as a separate entity.MEITL patients,predominantly male,typically present with vague and nonspecific symptoms and diagnosis is predominantly confirmed at laparotomy.Currently,there are no standardized treatment protocols,and the optimal therapy remains unclear.CASE SUMMARY We report a case of MEITL that was initially considered to be gastrointestinal stromal tumor(GIST)and Imatinib was administered for one cycle.The 62-yearold man presented with abdominal pain,abdominal distension,and weight loss of 20 pounds.Within 2 wk,the size of the mass considerably increased on computed tomography scans.The patient underwent surgery followed by chemotherapy with CHOP(cyclophosphamide,doxorubicin,vincristine,and prednisone)and stem-cell transplant.A correct diagnosis of MEITL was established based on postoperative pathology.Immunophenotypically,the neoplastic cells fulfilled the diagnostic criteria for MEITL as they were CD3+,CD4+,CD8+,CD56+,and TIA-1+.CONCLUSION Given that MEITL has no predisposing factor and presents with vague symptoms with rapid progression,the concomitant presence of abdominal symptoms and B symptoms(weight loss,fever,and night sweats)with hypoalbuminemia,anemia,low lymphocytic count and endoscopic findings of diffuse infiltrating type lesions should alert physicians to this rare disease,especially when it comes to Asian patients.Immediate laparotomy should then be carried out followed by chemotherapy and stem-cell transplant.

Refractory celiac disease and sprue-like intestinal disease

Celiac disease is a gluten-dependent small intestinal mucosal disorder that causes malabsorption, often with diarrhea and weight loss. Diagnosis is based on detection of typical biopsy changes in the proximal small bowel, followed by evidence for an unequivocal response to a gluten-free diet. Refractoriness in celiac disease may be due to poor diet compliance, sometimes intentional, or consumption of ubiquitious sources of gluten. Alternatively, the original diagnosis may not be correct (eg., duodenal Crohn's disease), or a second cause for symptoms may be present (eg., collagenous colitis, functional bowel disorder). In some with recurrent symptoms, a complication may be present (eg., collagenous sprue, small bowel carcinoma, lymphoma). In some, a response to a gluten-free diet can not be unequivocally defined, and more precise historical terms have been used including "sprue-like intestinal disease" or "unclassified sprue". Although a "wastebasket diagnosis", these likely represent a heterogeneous group, and some, but not all, may develop lymphoma. Precise definition will be critical in the future as an array of new treatments, including biological agents, may emerge.

Association between intestinal neoplasms and celiac disease:A review

Celiac disease(CD)is a chronic immune-mediated intestinal disease with genetic susceptibility.It is characterized by inflammatory damage to the small intestine after ingestion of cereals and products containing gluten protein.In recent years,the global prevalence rate of CD has been approximately 1%,and is gradually increasing.CD patients adhere to a gluten-free diet(GFD)throughout their entire life.However,it is difficult to adhere strictly to a GFD.Untreated CD may be accompanied by gastrointestinal symptoms,such as diarrhea,abdominal pain,and extraintestinal symptoms caused by secondary malnutrition.Many studies have suggested that CD is associated with intestinal tumors such as enteropathyassociated T-cell lymphoma(EATL),small bowel cancer(SBC),and colorectal cancer.In this study,we reviewed related studies published in the literature to provide a reference for the prevention and treatment of intestinal tumors in patients with CD.Compared with the general population,CD patients had a high total risk of SBC and EATL,but not colorectal cancer.The protective effect of GFD on CD-related malignancies is controversial.Further studies are needed to confirm whether GFD treatment can reduce the risk of intestinal neoplasms in CD.

A case of colon perforation due to enteropathy-associated T-cell lymphoma

Enteropathy-associated T-cell lymphoma (EATL) is an extremely rare disease, which is often related to gluten-sensitive enteropathy. It is an uncommon intestinal lymphoma with very poor prognosis and high mortality rate. In the absence of specific symptoms or radiological findings, it is difficult to diagnose early. Major complications of EATL have been known as intestinal perforation or obstruction, and only 5 cases of EATL are reported in South Korea. In this study, we report a case of 71-year-old male with symptoms of diarrhea, which later it progressed into cancer perforation of the colon. The initial colonoscopic findings were normal and computed tomography scan demonstrated a segmental wall thickening of the distal ascending colon with nonspecific multiple small lymphnodes, along the ileocolic vessels, but no signs of mass or obstruction. The histologic findings of resected specimen confirmed EATL type II. Patient expired two weeks after the operation. Therefore, we emphasize the need of random biopsy in the presence of normal mucosa appearance on colonoscopy for the early diagnosis of EATL.

Features of intestinal T-cell lymphomas in Chinese population without evidence of celiac disease and their close association with Epstein-Barr virus infection

Background Intestinal T-cell lymphoma （ITCL） is a heterogeneous lymphoid neoplastic group with variable clinical and pathological features. ITCL in oriental countries is different from enteropathy-type intestinal T-cell lymphoma （ETCL） in relation to celiac disease and Epstein-Barr virus （EBV）. The objective of this study was to investigate the clinicopathological features, immunophenotype, expression of cytotoxic molecule （TIA-1）, T-cell receptor （TCR）-γ gene rearrangement, and Epstein-Barr virus （EBV） latent infection in primary ITCL without celiac disease in Chinese. Methods The clinical data of 42 patients were analyzed, and the patients were followed up. Compared with human reactive lymphoid tissues, in situ hybridization for EBER1/2, polymerase chain reaction for TCR-~/gene rearrangement, and immunohistochemical staining for immunophenotypes, TIA-1 and EBV latent membrane proteins （LMP-1） were investigated. Survival curves of different clinicopathological features, immunophenotypes, expression of LMPI , TCR-γ/gene rearrangement and therapy were analyzed. Results Three fourths of the patients suffered from ITCL in China were men with a peak age incidence in the 4th decade. Common presenting features included fever and hemotochezia. The prognosis was poor with a median survival of 3.0 months. The lesions were mostly localized in the ileocecum and colon. About 38/42 （90. 5% ） patients demonstrated pleomorphic medium-sized on large seen. All 42 patients with ITCL revealed CD45RO cells. Histological features of celiac disease were rarely positive. Neoplastic cells partially expressed T-cell differentiated antigens （CD3ε, CD4, CD8） and NK cell associated antigen （CD56）. The positive frequency of CD3e, CIM, CD8 and CD56 was 28/42 （66.7%） 7/42 （16.7%）, 10/42 （23.8%） and 12/42 （28.6%） respectively. Thirty-nine cells （92. 9% ） expressed TIA-1, but none expressed CD20 and CD68. More than half of the patients （64. 3% , 64.3% and 59.5% ） revealed TCR-γ primers respectively. EBER1/2 was detected in 41 （97.6%） of LMP-1 was 38. 1% （16/42）. gene rearrangement by three different TCR-γ the 42 patients. The expression frequency ofConclusions Primary ITCL without celiac disease in Chinese is a special highly EBV-associated clinicopathological entity. There are few similarities in patients with celiac disease in western countries. A small proportion of primary ITCLs in Chinese and extranodal NK/T-eell lymphoma of nasal type belong to the same speetrum.

A case of rapid growing colonic NK/T cell lymphoma complicated by Crohn's disease

A 37-year-old man developed abdominal pain and bloody diarrhea 11 months before admission. The colonoscopy revealed multifocal ulcers in the colon. Histology showed active chronic inflammation. Although anti-tuberculosis medication was effective, his symptoms repeated 2 months later. The subsequent colonoscopy revealed more extensive irregular ulcers than before, and he was clinically suspected with intestinal malignant lymphoma. He underwent subtotal colectomy and was histologically suggested Crohn＇s disease, then 5-aminosalicylic and a combination of prednisone and azathioprine were administered in succession postoperatively, but they achieved minimal relief of symptoms for a period of 7 months. The third colonoscopy showed a large irregular ulcer in the ileocolon stomas, and primary colonic NK/T cell lymphoma was diagnosed through histological and immunophenotypic studies. Malignant lymphoma should be taken into consideration when clinically diagnosed Crohn＇s disease was refractory to medication or when its clinical course became aggressive.

单形性嗜上皮性肠道T细胞淋巴瘤3例报道与文献复习

目的结合病例报道和文献复习探讨单形性嗜上皮性肠道T细胞淋巴瘤(MEITL)的临床病理特征,为诊断和治疗提供经验和借鉴。方法回顾性分析3例MEITL患者的临床表现、组织病理学和免疫组化特点、诊治经过和预后,同时对既往发表文献中的其他案例进行复习和总结。结果本文报道的3例患者中,2例患者为中老年男性,1例为青年女性。3例患者的病变部位均在小肠,其中女性患者同时出现皮肤、乳腺受累。临床表现主要为腹胀、腹痛和肠道穿孔;病理特征主要为全层肠壁被大小均一的肿瘤细胞弥漫性浸润。免疫组化检测显示肿瘤细胞表达T细胞标记CD3、CD8、CD56、T细胞胞浆内抗原(TIA-1)、CD4和CD5;Ki-67增殖指数为50%~70%。在治疗上,病例1先后接受CHOP方案和GDP方案治疗,于确诊2年后死亡。病例2接受CHOP方案化疗1个疗程,随后放弃治疗死亡。病例3放弃治疗自动出院失访。结合既往发表的文献可见,MEITL发病率低,诊断较困难,具有高度侵袭性,患者对现有治疗的反应均较差。结论 MEITL好发于小肠,少见、进展快,患者易发生肠道穿孔、预后差,其诊断需结合临床表现、病理学特征和免疫组化检测结果等进行综合判断。尽早手术明确诊断并积极化疗可改善预后。

Truth lies below: A case report and literature review of typical appearing polyps yet with an atypical diagnosis

BACKGROUND Enteropathy associated T-cell lymphoma(EATL) is a rare form of peripheral Tcell lymphoma and makes up less than 5% of gastrointestinal lymphomas. EATL can be divided into type 1 which is associated with celiac disease, and monomorphic epitheliotropic intestinal T-cell lymphoma(MEITL), formally type 2, which is not associated with celiac disease.CASE SUMMARY We present a 60-year-old African American female, without celiac disease, who presented with abdominal pain, diarrhea, and 30 lb. weight loss over a 3 month period. She was subsequently diagnosed with EATL throughout her entire gastrointestinal tract. She is currently undergoing chemotherapy with EOCH(Etoposide, Oncovin, Cyclophosphamide, and Hydroxydaunorubicin). EATL is most common in the Asian and Hispanic population yet the incidence in African Americans is uncertain and emphasizes the rarity of this case. A literature review was included to further emphasize similarities and differences between our case and previously reported cases of MEITL.CONCLUSION The patient was diagnosed with EATL, immunochemical testing was not conclusive for MEITL however was suggestive of the disease.