Aplastic anemia associated with Crohn's disease: a tertiary center retrospective study

Aplastic anemia associated with Crohn's disease: a tertiary center retrospective study
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再生障碍性贫血与克罗恩病相关:一项三级中心回顾性研究

DOI:
10.1007/s00277-019-03729-4
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发表时间:
2019
期刊:
Ann Hematol
影响因子:
--
通讯作者:
Liang J
Liang J
中科院分区:
其他
文献类型:
--
作者:
Su S;Liu Z;Wang F;Zhang Y;Chu Y;Jiang M;Xu B;Wu N;Lin J;Xue X;Shi Y;Yang S;Wu K;Liang J

文献摘要

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再生障碍性贫血(AA)被报道与炎症性肠病(IBD)有关,但主要与溃疡性结肠炎(UC)有关。关于再生障碍性贫血和克罗恩病(CD)之间的联系,人们知之甚少。我们的目标是确定AA-CD患者的肖像。对西京消化医院IBD中心2008-01/2018-10收治的657例CD患者进行复习。收集了这组患者的临床表现、病史、内窥镜特征、治疗反应和预后。确诊为再生障碍性贫血的6名男性患者。本组CD合并再障的发生率为0.91%。CD和AA的平均诊断年龄分别为41.5岁和39.2岁。腹痛和高热是最常见的症状。内窥镜检查显示不再出现严重炎症,所有患者均表现为回盲瓣畸形。常规药物治疗未能取得良好效果。6例患者中有4例死于CD进展及其并发症。这些患者都没有因为贫困而接受骨髓移植治疗。AA和CD并发是一种相对罕见的情况。免疫损伤可能起到重要的致病作用,值得进一步关注。男性更容易患上这种情况。AA-CD患者临床病程较重,预后较差。传统疗法没有取得有效的效果,异基因干细胞移植可能是一种潜在的有效疗法。
Aplastic anemia (AA) has been reported to be associated with inflammatory bowel disease (IBD), but mostly with ulcerative colitis (UC). Little is known about the associations between AA and Crohn’s disease (CD). We aim to determine the portraits of patients with AA-CD. Among a total of 657 patients with CD registered in Xijing Hospital of Digestive Diseases IBD center from January 2008 to October 2018, the patients diagnosed with concurrent AA were reviewed. Clinical presentation, medical history, endoscopic features, response to treatment, and prognosis in this set of patients were collected. Six male patients confirmed as CD associated with AA were identified. The incidence rate was 0.91% for CD associated with AA in our series. Average age at diagnosis of CD and AA was 41.5 and 39.2 years old, respectively. Abdominal pain and hyperpyrexia were the most common symptoms. Endoscopic findings showed discontinued severe inflammation, and all these patients presented with deformed ileocecal valve. Conventional pharmacotherapy failed to achieve a favorable effect. Four of six patients died from CD progression and its complications. None of these patients received bone marrow transplantation treatment because of poverty. Concurrence of AA and CD is a relatively rare condition. Immunologic impairment may play an important pathogenic role and deserves further attention. Males are more susceptible to this condition. Patients with AA-CD are prone to a severe clinical course and poor prognosis. Conventional therapy achieves no potent effect, and allogeneic stem cell transplantation may be a potentially efficient therapy.