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 glutensensitive 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.

Early colon cancer with enteropathy-associated T-cell lymphoma involving the whole gastrointestinal tract:A case report

BACKGROUND Enteropathy-associated T-cell lymphoma(EATL)is a rare invasive lymphoma derived from gastrointestinal epithelial T lymphocytes.EATL involving the whole gastrointestinal tract accompanied with early colon cancer is extremely rare.CASE SUMMARY We present the case of a 67-year-old man with diarrhea for more than 5 mo whose colonoscopy in another hospital showed multiple colonic polyps,which indicated moderate to severe dysplasia and focal early cancer.Therefore,he was referred to our hospital for further endoscopic treatment.Colonoscopy after admission showed that the mucosa of the terminal ileum and the entire colon were slightly swollen and finely granular.Endoscopic mucosal resection was performed for colonic polyps located in the liver flexure of the colon and descending colon,respectively.Histopathological findings revealed diffuse infiltration of mediumsized lymphoid cells in the colonic mucosa and visible lymphoepithelial lesions.The histopathology of the polyp in the descending colon indicated moderately differentiated adenocarcinoma limited to the mucosa with negative resection margins.Additionally,immunohistochemical analysis showed positive staining for CD7 and CD8.Therefore,we arrived at a diagnosis of EATL with early colon cancer.Subsequently,the patient was transferred to the hematology department for chemotherapy.The patient’s diarrhea was not significantly relieved after receiving chemotherapy,and he ultimately died of severe myelosuppression.CONCLUSION EATL should be considered in unexplained chronic diarrhea.EATL progresses rapidly with a poor prognosis,especially when accompanied with early colon cancer.

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.METHODS:Sixty-eight patients who were diagnosed with ITCL in case reports in the Chinese literature were compiled and reviewed.Age,gender,CD56 expression,surgical management,multifocal nature,perforation and cyclophosphamide chemotherapy were analyzed as the prognostic factors.The Kaplan-Meier method was adopted for the univariate analysis and the cumulative survival curve analysis.RESULTS:The male-to-female ratio was 1.52 to 1.The median age was 41.7 years.Twenty-seven patients had symptoms of abdominal pain or diarrhea.Thirty-six of60 patients with temperature records had high fevers at the onset of the illness.Twenty-six of 34 patients who underwent fiberoptic colonoscopy were misdiagnosed with Crohn’s disease,intestinal tuberculosis or cancer.Sixty-one patients underwent surgery.Twelve of 61 surgical patients required a second operation for anastomotic leakage or secondary perforation.The sites of lesion involvement were the jejunum(8.82%),ileum(29.41%),ileum and colon(4.41%),colon(55.88%)and appendix(1.47%).The median cumulative survival rate was 3 mo(3.00±0.48).CONCLUSION:Efforts should be made to correctly diagnose ITCL and select the proper operative approach that may reduce serious complications and create opportunities for further treatment.

Multiple cerebral lesions in a patient with refractory celiac disease:A case report

BACKGROUND Enteropathy-associated T cell lymphoma(EATL)is an aggressive intestinal T cell lymphoma derived from intraepithelial lymphocytes,which occurs in individuals with celiac disease(CD).Cerebral involvement is an extremely rare condition and as described so far,lymphoma lesions may present as parenchymal predominantly supratentorial or leptomeningeal involvement.We describe a case of EATL with multifocal supra-and infratentorial brain involvement in a patient with refractory celiac disease(RCD).CASE SUMMARY A 58-years old man with known CD developed ulcerative jejunitis and was diagnosed with RCD type II.Six months later he presented with subacute cerebellar symptoms(gait ataxia,double vision,dizziness).Cranial magnetic resonance imaging(MRI)revealed multifocal T2 hyperintense supra-and infratentorial lesions.Laboratory studies of blood and cerebrospinal fluid were inconspicuous for infectious,inflammatory or autoimmune diseases.18Ffluorodeoxyglucose-positron emission tomography/computed tomography(18FDG-PET/CT)scan showed a suspect hypermetabolic lesion in the left upper abdomen and consequent surgical jejunal resection revealed the diagnosis of EATL.During the diagnostic work-up,neurological symptoms aggravated and evolved refractory to high-dosage cortisone.Recurrent MRI scans showed progressive cerebral lesions,highly suspicious for lymphoma and methotrexate chemotherapy was initiated.Unfortunately,clinically the patient responded only transiently.Finally,cerebral biopsy confirmed the diagnosis of cerebral involvement of EATL.Considering the poor prognosis and deterioration of the performance status,best supportive care was started.The patient passed away three weeks after diagnosis.CONCLUSION EATL with cerebral involvement must be considered as a possible differential diagnosis in patients with known RCD presenting with neurological symptoms.

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.

Possible relationship between refractory celiac disease and malignancies

Celiac disease(CeD)is a chronic autoimmune disorder that is triggered by gluten in genetically susceptible individuals,and that is characterized by CeD-specific antibodies,HLA-DQ2 and/or HLA-DQ8 haplotypes,enteropathy and different clinical pictures related to many organs.Intestinal lymphoma may develop as a result of refractory CeD.If a patient diagnosed with CeD is symptomatic despite a strict gluten-free diet for at least 12 months,and does not improve with severe villous atrophy,refractory CeD can be considered present.The second of the two types of refractory CeD has abnormal monoclonal intraepithelial lymphocytes and can be considered as pre-lymphoma,and the next picture that will emerge is enteropathy-associated T-cell lymphoma.This manuscript addresses"CeD and malignancies"through a review of current literature and guidelines.