Fontan 相关蛋白丢失性肠病与黏膜相关淋巴组织（MALT 淋巴瘤）的淋巴发育不良重叠：对蛋白丢失性肠病治疗的影响。
Overlap of lymphatic dysplasia in Fontan-associated protein-losing enteropathy and Mucosa-Associated Lymphoid Tissue (MALT lymphoma): implications for management of protein-losing enteropathy.
机构信息
Division of Pediatric Cardiology, University of Michigan, Ann Arbor, MI, USA48109.
Division of Pediatric Hematology and Oncology, University of Michigan, Ann Arbor, MI, USA48109.
出版信息
Cardiol Young. 2020 Dec;30(12):1973-1975. doi: 10.1017/S1047951120003182. Epub 2020 Oct 5.
Lymphatic vessel dysplasia is associated with Fontan-associated protein-losing enteropathy. Extra nodal non-Hodgkin lymphomas including mucosa-associated lymphoid tissue (MALT lymphoma) are associated with lymphatic vessel dysplasia. Here, we describe the case of a 7-year-old with Fontan-associated protein-losing enteropathy who developed MALT lymphoma with a clinical course indicative of interaction between these pathologies and improvement in protein-losing enteropathy after MALT lymphoma treatment. This case suggests a pathophysiologic overlap which has implications for the management of Fontan-associated protein-losing enteropathy.
淋巴血管发育不良与法乐四联症相关蛋白丢失性肠病相关。结外非霍奇金淋巴瘤包括黏膜相关淋巴组织（MALT 淋巴瘤）与淋巴血管发育不良相关。在此，我们描述了一例 7 岁法乐四联症相关蛋白丢失性肠病患儿，其发展为 MALT 淋巴瘤，临床表现提示这些病理之间存在相互作用，并且在 MALT 淋巴瘤治疗后蛋白丢失性肠病得到改善。该病例提示了一种病理生理学重叠，这对法乐四联症相关蛋白丢失性肠病的治疗具有重要意义。
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