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...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.展开更多
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 lym...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.展开更多
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.Adva...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.展开更多
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 cel...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.展开更多
基金Supported by Zhejiang Province Traditional Chinese Medicine Science and Technology Project,No.2024ZL1296.
文摘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.
文摘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.
文摘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.
文摘目的 探讨单形性嗜上皮性肠道T细胞淋巴瘤(MEITL)的临床特点及分期、病理学表现,以期提高临床医生对该病的认识。方法 回顾性分析2020年1月至2022年1月浙江大学医学院附属第一医院血液科收治的7例MEITL患者。收集整理患者基本人口学资料、临床表现、病理学结果、EBER原位杂交及TCR基因重排等分子生物学检测。分析MEITL的临床、病理特征。结果 7例患者中男2例,女5例;年龄55~67岁,中位年龄64岁;病程1~6个月,中位数2个月;腹痛6例,1例腹泻合并低钾血症起病,无乳糜泻。发病部位小肠4例,十二指肠、空肠及结肠各1例。乳酸脱氢酶升高3例,轻度贫血2例。癌胚抗原均未见升高。糖类抗原125、糖类抗原19-9及血清铁蛋白异常升高2例。7例骨髓学检查均无侵犯。3例在手术前行PET-CT检查,见受累处18F-氟脱氧葡萄糖(FDG)明显摄取增高,SUVmax波动在15.20~18.46。Lugano分期Ⅱ-1期1例、Ⅲ期1例、Ⅳ期5例。T细胞淋巴瘤预后指标(PIT)2分1例、3分6例。2例为内镜活检、5例行外科手术病理证实。6例行外科肠肿物切除手术,其中3例因继发小肠肠穿孔或梗阻行急诊手术,3例加结肠病变部分切除,肿瘤直径5~10 cm 1例,>10 cm 2例。光镜下瘤细胞呈单一性、大小为小至中等。免疫表型:CD3阳性(7/7)、CD8阳性(7/7)、CD56阳性(6/7)、TIA-1阳性(5/7);CD5阴性(6/6)、CD30阴性(5/5);1例CD20反常阳性表达。Ki-67指数中位数为70%,7例EBER阴性,2例TCR基因重排阳性。行基因检测1例:KRAS、FAT1、KMT2A、KMT2B、SETD2基因高频突变。7例均行化疗,包括为CHOP、AZA+CHOP、ECHOP、GVD联合西达苯胺等方案;1例行自体干细胞移植。7例均得到随访(末次随访时间2022年1月),死亡4例,存活3例。结论 MEITL罕见,高度恶性,进展迅速,无特异的临床表现,病死率高,基因重排等分子生物学检测是重要手段。
文摘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.
