Gastrointestinal (GI) tract involvement in Langerhans cell histiocytosis (LCH) is extremely rare. Langerhans cell histiocytosis with GI tract involvement (GI-LCH) is frequently associated with multi-system disease, and usually presents with severe systemic symptoms, such as protein-losing enteropathy (PLE). Although the GI tract is not included among the organs at risk, the prognosis of GI-LCH is poor, and no effective chemotherapeutic regimen has been identified. Here, we report an infant case of primary refractory GI-LCH with PLE that showed marked improvement in response to 2-chlorodeoxyadenosine (2-CdA) therapy with no severe adverse events, even under conditions of deteriorating general health. The present findings indicate that 2-CdA may be effective for refractory GI-LCH with PLE. Further studies are warranted to determine the optimal therapeutic strategies for GI-LCH with PLE.

中文翻译：

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用2-氯脱氧腺苷成功治疗顽固性小儿朗格汉斯细胞组织细胞增生症，并初步累及胃肠道。

胃肠道（GI）参与朗格汉斯细胞组织细胞增生症（LCH）极为罕见。胃肠道受累（GI-LCH）的朗格汉斯细胞组织细胞增生症经常与多系统疾病相关，并且通常表现为严重的全身症状，例如蛋白质丢失性肠病（PLE）。尽管胃肠道不包括在有风险的器官中，但是GI-LCH的预后很差，并且尚未确定有效的化疗方案。在这里，我们报道了一名婴儿，患有PLE的原发性难治性GI-LCH婴儿病例，即使在一般健康状况恶化的情况下，对2-氯脱氧腺苷（2-CdA）治疗的反应也明显改善，没有严重的不良事件。目前的发现表明2-CdA对于PLE治疗难治性GI-LCH可能有效。