Kircher Sheetal M, Gurbuxani Sandeep, Smith Sonali M
Department of Internal Medicine, University of Chicago Hospitals, 5841 S. Maryland Avenue, MC 2115, Chicago, IL 60637, USA.
J Gastrointest Cancer. 2007;38(1):19-23. doi: 10.1007/s12029-008-9009-z.
Enteropathy-associated T-cell lymphoma (EATL) is a rare subtype of non-Hodgkin's lymphoma characterized by an aggressive phenotype and poor outcome in the vast majority of cases. Substantial portions of patients have either no prior diagnosis of celiac disease or have a subacute course of celiac disease followed by EATL diagnosis. We report a case of a 72-year-old African American male without history of gastrointestinal intolerance who presented with acute abdominal pain and weight loss leading to the eventual diagnosis of gastric EATL. Despite an initial promising clinical and radiographic response to cyclophosphamide, doxorubicin, vincristine, and prednisone plus alemtuzumab, the disease rapidly progressed with a fatal outcome. This case and review of the literature highlights the features of this uncommon disease and addresses both the diagnostic and therapeutic challenges of this aggressive malignancy. We also discuss our experience with the use of fluoro-2-deoxy-D-glucose positron emission tomography in monitoring treatment response.
肠病相关T细胞淋巴瘤（EATL）是一种罕见的非霍奇金淋巴瘤亚型，其特征是在绝大多数病例中具有侵袭性表型和不良预后。相当一部分患者既往没有乳糜泻诊断，或者有亚急性乳糜泻病程，随后被诊断为EATL。我们报告一例72岁非裔美国男性，无胃肠道不耐受病史，因急性腹痛和体重减轻就诊，最终诊断为胃EATL。尽管最初对环磷酰胺、阿霉素、长春新碱、泼尼松加阿仑单抗有良好的临床和影像学反应，但疾病迅速进展，导致致命结局。该病例及文献复习突出了这种罕见疾病的特征，并探讨了这种侵袭性恶性肿瘤的诊断和治疗挑战。我们还讨论了使用氟代脱氧葡萄糖正电子发射断层扫描监测治疗反应的经验。
