Clinical Diagnosis and Treatment of Enteropathy-associated T-cell Lymphoma

Abstract

To investigate the clinical characteristics of enteropathy-associated T-cell lymphoma(EATL) and to give treatment strategies. METHODS:  From 2019. 02-2022. 02, 10 patients with enteropathy-associated T-cell lymphoma were seen in our hospital, their clinical data were retrospectively analyzed, and practical treatment plans were provided. Results:None of the patients had a history of intestinal disease and all presented to the hospital because of symptoms occurring in the gastrointestinal tract.Five patients had intestinal perforation and underwent dissection, three had small bowel masses and underwent surgery, and two had T-cell lymphoma and underwent related treatment. Six patients had lesions in the small intestine and the others had lesions in the rectum. According to AnnArbor clinical d staging, 9 patients(90%) had stage I+II and 1 patient(10%) had stage III. Gastrointestinal lymphoma AnnArbor clinical staging was the same as lugano clinical staging. 6 patients with B symptoms, 6 with physical status score ≥ 2, 5 with IPI > 2, 6 with PIT > 6; all patients had normal examination regardless of bone marrow examination, β2-microglobulin, or glycoconjugate antigen-125, erythrocyte sedimentation rate. 1 patient with anemia, 6 with decreased albumin, 2 with with elevated lactate dehydrogenase, and 1 case with increased C-reactive protein. According to immunohistochemical hints, 6 cases were CD56 positive and 4 in situ hybridization EBER positive; in the mid-term evaluation of combined patients, the complete remission(CR) rate was 90% in 10 patients; in the DA-EPOCH regimen, the complete remission(CR) rate was 80% in 10 patients. At the completion of 6 chemotherapy courses, all chemotherapy patients were in complete remission(CR) at the end of the period assessment, but 3 of them relapsed within one year, and the disease could not be controlled with the implementation of second-line regimen and chemotherapy again. The follow-up period was 1-25 months, with a high follow-up rate of 100% up to the follow-up time, and 6 patients died, with an overall 2-year survival rate of 40%. Conclusion: Enteropathy-associated T-cell lymphoma is highly aggressive, and after chemotherapy, patients can be treated but are prone to relapse. The use of allogeneic hematopoietic stem cell transplantation(ASCT) becomes the first-line consolidation therapy, which cannot obtain long-term efficacy and needs to be explored.