Boccon-Gibod L A, Krichen H A, Carlier-Mercier L M, Salaun J F, Fontaine J L, Leverger G R
Pathology Department, Hôpital d'Enfants Armand Trousseau, Paris, France.
Pediatr Pathol. 1992 Jul-Aug;12(4):515-24. doi: 10.3109/15513819209024201.
Protein-losing enteropathy was observed in two children with Langerhans' cell histiocytosis (LCH). One patient was an infant with congenital cutaneous lesions; the second child had sigmoid and lymph node infiltration. Electron microscopy and immunohistochemistry confirmed, in both, infiltration of duodenum, skin, and liver by LCH. Gastrointestinal involvement by LCH seldom produces prominent clinical manifestations but indicates widespread multisystem disease. Immunohistochemical and/or ultrastructural features allow definitive diagnosis from mucosal biopsy specimens. Review of the literature of gastrointestinal infiltration by LCH emphasizes its poor prognosis, especially when associated with organ dysfunction.
在两名朗格汉斯细胞组织细胞增多症（LCH）患儿中观察到蛋白丢失性肠病。一名患者为患有先天性皮肤病变的婴儿；第二名儿童有乙状结肠和淋巴结浸润。电子显微镜检查和免疫组织化学证实，两名患者的十二指肠、皮肤和肝脏均有LCH浸润。LCH累及胃肠道很少产生明显的临床表现，但提示广泛的多系统疾病。免疫组织化学和/或超微结构特征可从黏膜活检标本中做出明确诊断。对LCH胃肠道浸润的文献回顾强调其预后不良，尤其是与器官功能障碍相关时。
