Bagdi Eniko, Kiss József, Borbényi Zita, Piukovics Klára, Krenács László
Daganatpatológiai és Molekuláris Diagnosztikai Laboratórium, Szeged.
Orv Hetil. 2008 May 25;149(21):995-8. doi: 10.1556/OH.2008.28306.
Refractory sprue is characterised by distinctive morphologic alterations and the emergence of clonal intraepithelial lymphocytes.
In this case report the authors emphasize the importance of histopathology in the diagnosis of refractory sprue.
The sequential biopsies from this patient have been investigated with routine histology, immunohistochemistry and molecular genetics for T-cell clonality analysis.
The severely cachectic patient presenting with malabsorption syndrome has been diagnosed with celiac disease through a duodenal biopsy, and the CD8 negativity of the intraepithelial lymphocytes suggested the possible diagnosis of refractory sprue. Azathioprine and glucocorticoid therapy was administered due to the failed jejunal feeding and gluten-free diet, resulting in clinically complete, morphologically partial remission. Intestinal T-cell lymphoma developed in the ileocecal region within two years after the first clinical presentation.
Refractory sprue and the enteropathy-type T-cell lymphoma constitute a disease spectrum. The reported case shows how a simple method can provide crucial information in the diagnosis of refractory sprue.
难治性口炎性腹泻的特征为独特的形态学改变及克隆性上皮内淋巴细胞的出现。
在本病例报告中，作者强调了组织病理学在难治性口炎性腹泻诊断中的重要性。
对该患者的系列活检组织进行了常规组织学、免疫组织化学及分子遗传学检查，以分析T细胞克隆性。
该严重恶病质且伴有吸收不良综合征的患者经十二指肠活检被诊断为乳糜泻，上皮内淋巴细胞CD8阴性提示可能为难治性口炎性腹泻。由于空肠喂养及无麸质饮食失败，给予硫唑嘌呤和糖皮质激素治疗，临床完全缓解，但形态学部分缓解。首次临床表现后两年内，回盲部发生了肠T细胞淋巴瘤。
难治性口炎性腹泻和肠病型T细胞淋巴瘤构成一个疾病谱。报告的病例显示了一种简单方法如何能在难治性口炎性腹泻的诊断中提供关键信息。
