Gastrointestinal LCH: a rare manifestation of Langerhans cell histiocytosis

Introduction: Langerhans cell histiocytosis (LCH) is a rare neoplasm marked by the proliferation of Langerhans cells, primarily affecting children under 2 years old. Gastrointestinal (GI) involvement in LCH is uncommon and often part of widespread disease.

Case presentation: We report a 16-year-old female with a history of LCH, previously treated with 6-mercaptopurine and vinblastine, who presented with bloody diarrhoea, abdominal pain, and vomiting. Examination revealed hypopigmented skin lesions, lymphadenopathy, and hepatosplenomegaly. Laboratory tests indicated anaemia and eosinophilia, and colonoscopy was suggestive of GI LCH.

Discussion: Gastrointestinal LCH often presents with nonspecific symptoms. It is crucial to maintain a high degree of suspicion for GI LCH in atypical GI presentations, as treatment outcomes can be challenging if diagnosed late or misdiagnosed.

Conclusion: GI symptoms in LCH are rare but may occur in isolation. Early diagnosis and treatment are crucial to reduce morbidity and improve prognosis.