Keio University School of Medicine, Department of Pediatrics, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan.
Pediatrics. 2010 Feb;125(2):e426-32. doi: 10.1542/peds.2009-1742. Epub 2010 Jan 18.
Protein-losing enteropathy (PLE) is frequently complicated in patients with gastrointestinal tract-involved Langerhans cell histiocytosis (LCH); however, LCH per se is not generally included in the list of diseases that cause PLE. We report here a case of infantile PLE that presented with continuous diarrhea at the onset of LCH. She was initially diagnosed as having allergic gastroenteropathy and, thus, received intravenous prednisolone, which was thought to have induced immunodeficiency and consequently resulted in life-threatening cytomegalovirus-associated hemophagocytic syndrome and disseminated intravascular coagulation. Because chemotherapy for hemophagocytic syndrome was transiently effective for underlying LCH as well, the diagnosis of LCH was delayed until its recurrence. Gastrointestinal tract-involved LCH, a rare but highly fatal disease, should be considered for infants with refractory gastrointestinal symptoms, especially for those with PLE; endoscopic biopsy is strongly recommended for immediate diagnosis.
蛋白丢失性肠病（PLE）常并发于胃肠道受累的朗格汉斯细胞组织细胞增生症（LCH）患者；然而，LCH 本身通常不包括在引起 PLE 的疾病列表中。我们在此报告一例婴儿 PLE 病例，其在 LCH 发病时出现持续性腹泻。她最初被诊断为过敏性胃肠病，并因此接受了静脉注射泼尼松龙，这被认为导致了免疫缺陷，进而导致危及生命的巨细胞病毒相关噬血细胞综合征和弥漫性血管内凝血。由于噬血细胞综合征的化疗对潜在的 LCH 也有短暂的疗效，因此 LCH 的诊断被延迟到其复发。胃肠道受累的 LCH 是一种罕见但高度致命的疾病，对于有难治性胃肠道症状的婴儿，尤其是有 PLE 的婴儿，应考虑该病；强烈建议进行内镜活检以立即诊断。
