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Abstract: This case involves a 6-month-old male infant who has been admitted to the hospital repeatedly due to diarrhea since the age of 2 months. Later，the diarrhea gradually worsened，accompanied by manifestations of protein-losing enteropathy（PLE） such as fever and edema. The patient visited Beijing Children’s Hospital Affiliated to Capital Medical University on May 22，2025，and a multidisciplinary team（MDT） conducted joint diagnosis and treatment. The final confirmed diagnosis was Langerhans cell histiocytosis（LCH）. After treatment with targeted anti-tumor drugs and symptomatic therapy，the infant’s condition improved. The core challenge of this case lies in the clinical rarity of LCH presenting with gastrointestinal symptoms as the initial manifestation. For children with diarrhea，hematochezia，and fever that respond poorly to conventional treatment，especially those with multisystem damage，early gastrointestinal mucosal pathology combined with special immunohistochemical tests should be performed based on clinical manifestations to confirm the diagnosis as soon as possible. Attention should be paid to strengthening inter-departmental communication and administering specialized treatment at an early stage to improve the prognosis of such children.

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