Am J Hematol：乳糜泻病人更可能患上淋巴细胞增生性疾病

根据一项发表在2012年8月那期American Journal of Hematology期刊上的研究，患有乳糜泻(celiac disease)的病人，特别是那些在生命后期患上吸收不良症(malabsorption symptom)那些病人，有更高的淋巴增生性疾病(lymphoproliferative disorder, LPD)发生率。

来自美国哥伦比亚大学医学中心的Lori A. Leslie博士和同事们开展一项涉及1285名患有乳糜泻的成年人的回顾性群体研究以便确定LPD亚型发生率和不同LPD亚型的存活时间。

研究人员鉴定出40名病人患上LPD(标准化发病比[standardized incidence ratio, SIR]为6.48)，其中33名患上非何杰金淋巴瘤(non-Hodgkin's lymphoma, NHL; SIR为6.91)。当患上LPD之前，只考虑确诊为乳糜泻的那些病人时，肠病相关性T细胞(enteropathy-associated T-cell, EATL)、非EATL T细胞(non-EATL T-cell)、弥漫性大B细胞(diffuse large B-cell)、套细胞(mantle cell)和边缘区(marginal zone)非何杰金淋巴瘤亚型发生率与乳糜泻的关联性显著提高。

相对于患上非EATL的病人，患上 EATL的病人平均存活时间更短。患上LPD的病人更可能发生腹泻、腹痛和/或体重减轻，而且被真多为乳糜泻的时间更晚(由41.4岁上升至57.9岁)。

研究人员写道，“在未来，我们还需进一步分析乳糜泻和不同LPD亚型之间的关联性以便鉴定出炎症途径和淋巴增殖途径中的相同分子，并将它们作为大有希望的靶标以便用于药物开发。这些发现可能被用来对病人患上淋巴瘤的风险进行分类，设计监测方案，并给有更高风险患上LPD的乳糜泻病人提出预防性策略。”

本文编译自Celiac disease linked to lymphoproliferative disorders

doi: 10.1002/ajh.23237
PMC:
PMID:

Incidence of lymphoproliferative disorders in patients with celiac disease

Lori A. Leslie1, Benjamin Lebwohl2, Alfred I. Neugut3,4, John Gregory Mears3, Govind Bhagat5, Peter H.R. Green

Prior studies describe an increased incidence of lymphoma in celiac disease. However, few studies differentiate among lymphoproliferative disorders (LPDs). Our aim was to determine incidences of LPD subtypes in celiac disease patients, describe patterns of celiac disease presentation in patients who develop LPD, and compare survival in patients with various LPD subtypes. We conducted a retrospective cohort study of adults with biopsy-proven celiac disease seen at a US referral center from 1981 to 2010, identified patients with comorbid LPD, and calculated standardized incidence ratios (SIR) for each LPD subtype. In our cohort of 1,285 patients with celiac disease, there were 40 patients with LPD [SIR = 6.48, 95% confidence interval (CI) = 4.62–8.64] including 33 with non-Hodgkin lymphoma (NHL, SIR = 6.91, 95% CI = 4.26–8.28). The incidences of NHL subtypes including enteropathy-associated T-cell (EATL, n = 12), non-EATL T-cell (SIR = 22.43, 95% CI = 7.08–46.41), diffuse large B-cell (SIR = 5.37, 95% CI = 1.93–10.52), mantle cell (SIR = 32.21, 95% CI = 6.07–78.97), and marginal zone (SIR = 37.17, 11.73–76.89) lymphoma remained significantly elevated when only those diagnosed with celiac before LPD were considered (n = 24, NHL SIR = 4.47, 95% CI = 2.86–6.44). Patients who developed LPD were older at time of celiac disease diagnosis (57.9 ± 15.5 versus 42.5 ± 17.4 years, P < 0.0001) and more likely to present with diarrhea (60.0% versus 39.8% P = 0.016), abdominal pain (17.5% versus 5.5% P = 0.0046), and/or weight loss (12.5% versus 4.0%, P = 0.028). EATL patients had a shorter average survival than non-EATL NHL patients (3.2 versus 15.0 years, P = 0.016). The incidence of LPD is increased in celiac disease patients. Those diagnosed later in life who present with symptoms of malabsorption are more likely to be diagnosed with LPD.