Egan L J, Walsh S V, Stevens F M, Connolly C E, Egan E L, McCarthy C F
Department of Medicine, University College Hospital, Galway, Ireland.
J Clin Gastroenterol. 1995 Sep;21(2):123-9.
Celiac sprue (CS) is frequently complicated by malignancy, most commonly small intestinal lymphoma. Our study was performed in an area with a high prevalence of CS to define the clinical features, response to treatment, and outcome of this tumor. Of a total of 31 lymphomas complicating CS identified, 30 case records and 24 tumor specimens were reviewed. Overall 1-year survival was 9 of 29 (31%) and 5-year survival 3 of 27 (11%). Seven previously diagnosed celiac patients developed lymphoma; length on gluten-free diet ranged from 12 to 252 months (median 44 months). In this group, presentation was nonspecific, diagnosis difficult, and survival poor (lymphoma diagnosed in life in four of seven, mean survival 2.25 months). Twenty-three patients had CS and lymphoma diagnosed during the same illness. In this group, 14 of 23 presented with a surgical emergency and were treated with tumor resection and chemotherapy. Nine are disease-free and alive or died of another cause after 10-196 months (mean 74 follow-up). Celiac-associated lymphoma is a frequent, difficult to diagnose, and commonly fatal complication of CS. An aggressive diagnostic approach, including laparoscopy, is recommended. Long-term survival can be expected in a significant number of these patients and in our series was almost exclusively confined to those treated with chemotherapy.
乳糜泻（CS）常并发恶性肿瘤，最常见的是小肠淋巴瘤。我们的研究在CS高发地区进行，以明确这种肿瘤的临床特征、对治疗的反应及预后。在总共31例并发CS的淋巴瘤中，回顾了30份病例记录和24份肿瘤标本。总体1年生存率为29例中的9例（31%），5年生存率为27例中的3例（11%）。7例先前诊断为乳糜泻的患者发生了淋巴瘤；无麸质饮食时间为12至252个月（中位时间44个月）。在这组患者中，临床表现不具特异性，诊断困难，生存率低（7例中有4例在生前诊断出淋巴瘤，平均生存2.25个月）。23例患者在同一疾病过程中被诊断出患有CS和淋巴瘤。在这组患者中，23例中有14例出现外科急症，接受了肿瘤切除和化疗。9例在10至196个月（平均随访74个月）后无病存活或死于其他原因。乳糜泻相关淋巴瘤是CS常见、难以诊断且通常致命的并发症。建议采用积极的诊断方法，包括腹腔镜检查。这些患者中有相当一部分有望获得长期生存，在我们的系列研究中，长期生存几乎仅局限于接受化疗的患者。
