乳糜泻和再生障碍性贫血的罕见关联。

IF 1.3 4区 医学 Q3 PATHOLOGY
Pediatric and Developmental Pathology Pub Date : 2025-07-01 Epub Date: 2025-03-29 DOI:10.1177/10935266251330162
Tatiana Moreira, Diana Simões, Fátima Ferreira, Eunice Trindade, Irene Carvalho
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引用次数: 0

摘要

乳糜泻(CD)是一种慢性免疫介导的疾病,由遗传易感个体摄入麸质引发。文献中已经报道了CD和再生障碍性贫血(AA)的关联,但这种关联在儿童中仍然很少见。作者报告了一例先前健康的4岁男孩,有1个月的腹泻、虚弱、食欲不振和体重减轻的病史。实验室评估显示双氧体减少症合并非常严重的再生性贫血和中性粒细胞减少症。骨髓抽吸和活检结果提示骨髓发育不全。进一步的病因学研究显示IgA缺乏和血浆抗组织转谷氨酰胺酶IgG抗体(抗ttg IgG 336 U/mL)浓度升高。患者接受了上消化道内窥镜检查,确认诊断为乳糜泻。该儿童开始无麸质饮食(GFD),随后临床和血清学改善。住院后随访12个月,患儿无症状,生长速度正常,双氧缺乏症消退,抗ttg IgG下降但仍呈阳性(151 U/ml),原因是GFD部分粘连。据作者所知,这是第8个已发表的描述乳糜泻与AA相关的儿科病例。这种关联的发病机制尚不完全清楚。作者建议,对于不明原因的血液学异常患者,应考虑进行乳糜泻筛查。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A Rare Association of Celiac Disease and Aplastic Anemia.

Celiac disease (CD) is a chronic immune-mediated disorder triggered by the ingestion of gluten in genetically predisposed individuals. Association of CD and aplastic anemia (AA) has been reported in the literature, yet this association remains rare in children. The authors report a case of a previously healthy 4-year-old boy with 1-month history of diarrhea, asthenia, loss of appetite, and weight loss. Laboratory evaluation showed bicytopenia with very severe aregenerative anemia and neutropenia. Bone marrow aspirate and biopsy were performed with findings suggestive of bone marrow aplasia. Further etiological research showed IgA deficiency and increased plasma concentrations of anti-tissue transglutaminase IgG antibodies (anti-tTG IgG 336 U/mL). Patient underwent upper digestive endoscopy confirming diagnosis of CD. The child started a gluten-free diet (GFD) with subsequent clinical and serological improvement. At 12-month post-hospitalization follow-up, the child was asymptomatic, with normal growth rate, resolution of bicytopenia, and anti-tTG IgG lower but still positive (151 U/ml) due to partial adhesion to GFD. To the best of author's knowledge, this is the eighth published pediatric case describing the association of CD with AA. The pathogenesis of this association is not yet fully understood. The authors suggest that CD screening should be considered in patients with unexplained hematological abnormalities.

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来源期刊
CiteScore
3.70
自引率
5.30%
发文量
59
审稿时长
6-12 weeks
期刊介绍: The Journal covers the spectrum of disorders of early development (including embryology, placentology, and teratology), gestational and perinatal diseases, and all diseases of childhood. Studies may be in any field of experimental, anatomic, or clinical pathology, including molecular pathology. Case reports are published only if they provide new insights into disease mechanisms or new information.
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