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红细胞谷氨酰胺转移酶抑制导致乳糜泻发病时溶血。

Erythrocytic transglutaminase inhibition hemolysis at presentation of celiac disease.

机构信息

Pediatric Clinic of the University Children's Hospital, Medical Faculty University of Belgrade, 10 Tiršova Street, Belgrade 11000, Serbia.

出版信息

World J Gastroenterol. 2010 Nov 28;16(44):5647-50. doi: 10.3748/wjg.v16.i44.5647.

DOI:10.3748/wjg.v16.i44.5647
PMID:21105201
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2992686/
Abstract

Celiac disease (CD) is a common autoimmune condition. Previously it was considered to be a rare childhood disorder, but is actually considered a relatively common condition, present at any age, which may have multiple complications and manifestations. Hematological disorders of the disease are not uncommon. Among these disorders, the most frequently reported are anemias as a result of iron deficiency, often associated with folate and/or B12 deficiency. Anemias caused by hemolysis are very rarely reported in celiac patients. An 11-year-old girl with a previous uneventful medical history presented with severe hemolytic anemia. Hemolysis was Coombs negative, accompanied by inappropriate low reticulocyte count, despite exaggerated bone marrow hyperplasia of the erythroid precursors which showed normal maturation. Serology for recent infections, including Epstein-Barr virus, parvovirus B19, cytomegalovirus and mycoplasma, were all negative. Levels of serum IgA, IgG and IgM, were all within normal ranges for age. Screening for anti-DNA, antinuclear, antineutrophil cytoplasmic, antimicrosomal, antithyroglobulin, and antimitochondrial antibodies and lupus anticoagulants, was negative. She was also negative for human immunodeficiency virus. Conventional therapy with corticosteroids and intravenous immunoglobulin failed. CD was serendipitously discovered upon screening for anti-tissue transglutaminase autoantibodies. The disease was confirmed by biopsy of the small intestine mucosa. The patient recovered with gluten-free diet. A unique case of CD is presented. CD should be serologically screened in each patient with Coombs negative "immune" hemolytic anemia, particularly if accompanied by "reticulocytopenia". A new hemolytic mechanism and very speculative explanation for "reticulocytopenia" are discussed.

摘要

乳糜泻(CD)是一种常见的自身免疫性疾病。以前它被认为是一种罕见的儿童疾病,但实际上被认为是一种相对常见的疾病,可发生于任何年龄,可能有多种并发症和表现。该疾病的血液学异常并不少见。在这些异常中,报告最多的是缺铁引起的贫血,常伴有叶酸和/或 B12 缺乏。溶血性贫血在乳糜泻患者中非常罕见。一名 11 岁女孩既往无特殊病史,表现为严重溶血性贫血。溶血为 Coombs 阴性,伴有不适当的低网织红细胞计数,尽管红系前体细胞的骨髓增生过度,但显示正常成熟。近期感染的血清学检查,包括 EB 病毒、细小病毒 B19、巨细胞病毒和支原体,均为阴性。血清 IgA、IgG 和 IgM 水平均在年龄正常范围内。抗 DNA、抗核、抗中性粒细胞胞质、抗微粒体、抗甲状腺球蛋白和抗线粒体抗体及狼疮抗凝物的筛查均为阴性。她也没有人类免疫缺陷病毒。皮质类固醇和静脉注射免疫球蛋白的常规治疗无效。在筛查抗组织转谷氨酰胺酶自身抗体时偶然发现 CD。通过小肠黏膜活检证实了该疾病。患者通过无麸质饮食恢复。报告了一例独特的 CD 病例。在 Coombs 阴性的“免疫”溶血性贫血患者中,应进行血清学筛查 CD,特别是伴有“网织红细胞减少症”的患者。讨论了一种新的溶血性机制和非常推测性的“网织红细胞减少症”解释。

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本文引用的文献

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Hematologic manifestations of celiac disease.乳糜泻的血液学表现。
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