摘要
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.
作者简介
Corresponding author:Lin-Bo Wang,Doctor,MBBS,MD,PhD,Attending Doctor,Chief Doctor,Director,Professor,Surgical Oncologist,Department of Surgical Oncology,Sir Run Run Shaw Hospital,Zhejiang University School of Medicine,No.3 Eastern Qingchun Road,Hangzhou 310006,Zhejiang Province,China.linbowang@zju.edu.cn,ORCID number:0000-0001-6594-8722;Abdul Saad Bissessur,ORCID number:0000-0003-4422-5511;Ji-Chun Zhou,ORCID number:0000-0002-0727-4034;Ling Xu,ORCID number:0000-0001-7259-4731;Zhao-Qing Li,ORCID number:0000-0001-7373-7562;Si-Wei Ju,ORCID number:0000-0001-5228-5384;Yun-Lu Jia,ORCID number:0000-0003-2213-7561。
