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作 者:Abdul Saad Bissessur Ji-Chun Zhou Ling Xu Zhao-Qing Li Si-Wei Ju Yun-Lu Jia Lin-Bo Wang
机构地区:[1]Department of Surgical Oncology,Sir Run Run Shaw Hospital,Zhejiang University School of Medicine,Hangzhou 310006,Zhejiang Province,China [2]Department of Medical Oncology,First Affiliated Hospital,Zhejiang University School of Medicine,Hangzhou 310006,Zhejiang Province,China
出 处:《World Journal of Gastrointestinal Oncology》2022年第11期2273-2287,共15页世界胃肠肿瘤学杂志(英文版)(电子版)
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.
关 键 词:Monomorphic epitheliotropic intestinal T-cell lymphoma Gastrointestinal stromal tumor Immunophenotypically CHEMOTHERAPY Stem-cell transplant Case report
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