Survival in refractory coeliac disease and enteropathyassociated T-cell lymphoma: retrospective evaluation of single-centre experience
Survival in refractory coeliac disease and enteropathyassociated T-cell lymphoma: retrospective evaluation of single-centre experience
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DOI：
10.1136/gut.2006.114512
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发表时间：
2007-10-01
期刊：
影响因子：
24.5
通讯作者：
Mulder, C. J. J.
中科院分区：
文献类型：
--
作者：
Al-Toma, A.;Verbeek, W. H. M.;Mulder, C. J. J.
背景：乳糜泻可能被认为是难治性疾病（RCD），当症状持续或复发，尽管严格遵守无麸质饮食。RCD可细分为I型和II型，分别具有表型正常和异常的上皮内t细胞群。RCD I似乎对硫唑嘌呤/强的松治疗反应良好。RCD II通常对任何已知的治疗都有耐药性，并且转变为肠病相关t细胞淋巴瘤（EATL）是常见的。目的：通过报道一大批复杂乳糜泻患者的长期生存和RCD转变为EATL的风险，进一步了解RCD和EATL的发展。设计与方法：回顾性比较四组合并乳糜泻患者对治疗的反应：43例，RCD I；50例RCD II（总），其中26例RCD II患者在一段时间对无麸质饮食不耐后发展为EATL（继发性EATL）， 13例EATL患者之前没有复杂的乳糜泻史（de novo EATL）。结果：在RCD I组中未发现与乳糜泻相关的死亡率。RCD I组的总5年生存率为96%；RCD II（总）组为58%；而在发展EATL后的RCD II组中，这一比例只有8%。新发EATL组的2年生存率为20%，而继发EATL组为15% （p = 0.63）。50例RCD II患者中有28例（56%）死亡，23例（46%）死于EATL， 4例死于进行性难治性消瘦，1例死于神经性乳糜泻。结论：值得注意的是，在平均5年随访期间（2 - 15年），没有RCD I患者发生RCD II或EATL。共有52%的RCD II患者在诊断为RCD II后的4 - 6年内发生了EATL。在RCD II和EATL中，更积极和有针对性的治疗似乎是必要的。
Background: Coeliac disease may be regarded as refractory disease (RCD) when symptoms persist or recur despite strict adherence to a gluten-free diet. RCD may be subdivided into types I and II with a phenotypically normal and aberrant intraepithelial T-cell population, respectively. RCD I seems to respond well to azathioprine/prednisone therapy. RCD II is usually resistant to any known therapy and transition into enteropathy-associated T-cell lymphoma (EATL) is common. Aim: To provide further insight into RCD and the development of EATL, by reporting on long-term survival and risk of transition of RCD into EATL in a large cohort of patients with complicated coeliac disease. Design andMethods: Retrospective comparison of responses to therapy in four groups of patients with complicated coeliac disease: 43, RCD I; 50, RCD II (total), of whom 26 with RCD II developed EATL after a period of refractoriness to a gluten-free diet (secondary EATL) and 13 were EATL patients without preceding history of complicated coeliac disease (de novo EATL).Results: No coeliac-disease-related mortality was recognised in the RCD I group. The overall 5-year survival in the RCD I group it was 96%; in the RCD II (total) group was 58%; and in the RCD II group after developing EATL it was only 8%. The 2-year survival in the de novo EATL group was 20% versus 15% in secondary EATL group (p = 0.63). Twenty-eight (56%) of the 50 patients with RCD II died, 23 (46%) due to EATL, 4 due to a progressive refractory state with emaciation and 1 from neurocoeliac disease.Conclusion: Remarkably, no patient with RCD I developed RCD II or EATL within the mean follow-up period of 5 years (range 2 - 15 years). A total of 52% of the RCD II patients developed EATL within 4 - 6 years after the diagnosis of RCD II. More aggressive and targeted therapies seem necessary in RCD II and EATL.