Gastrointestinal Tract Involvement of Langerhans Cell Histiocytosis: Concerning Point of Diagnosis

Abstract

An 8-month-old girl presented with chronic mucous bloody diarrhea for 3 months. She was diagnosed as infective gastroenteritis and did not improve after antibiotic treatment. Three months subsequently, she was diagnosed with allergic gastroenteritis after developing generalized skin rash and was treated with amino acid-based formula but the symptoms of diarrhea deteriorated. The results of a biopsy of the gastrointestinal tract and skin lesions confirmed the diagnosis of Langerhans cell histiocytosis. Although Langerhans cell histiocytosis is uncommon with gastrointestinal tract involvement, Langerhans cell histiocytosis should be listed in the differential diagnosis for patients who present with chronic mucous bloody diarrhea that is unresponsive to multimodality treatment.

Relapse of Non-Gastrointestinal Langerhans Cell Histiocytosis in the Rectum in a Child: A Case Report

“…Possible sites of disease include the skin, skeleton, liver, endocrine glands, spleen, central nervous system (CNS), thymus and lung. 2 , 7 , 8 Gastro-intestinal involvement is extremely rare, with only a few cases having been reported, worldwide, and only 1 case series being documented in the literature. 1 , 3 , 6 - 9 To the best of our knowledge, a total of 43 cases of paediatric intestinal LCH, had been published till 2021.…”

“… 2 , 7 , 8 Gastro-intestinal involvement is extremely rare, with only a few cases having been reported, worldwide, and only 1 case series being documented in the literature. 1 , 3 , 6 - 9 To the best of our knowledge, a total of 43 cases of paediatric intestinal LCH, had been published till 2021. 1 The literature suggests that gastro-intestinal LCH is usually associated with multisystem involvement, with a poor prognosis and a mortality rate of up to 55%.…”

“…Patients with GI involvement may present with variable symptoms, depending upon the area of the GI tract involved, including vomiting, abdominal pain, protein-losing enteropathy, bloody and non-bloody diarrhoea, malabsorption and failure to thrive. 2 - 4 In a case report by Chavananon et al, 8 the initial diagnosis of LCH in a child was missed because of the non-specific nature of the symptoms and almost all cases of GI LCH reported have had similar non-specific symptoms. 1 Our patient had a clear history of rectal bleeding and so was investigated early, with colonoscopy for this new-onset symptom on a background of previous disseminated LCH.…”

“…Owing to the rarity of LCH with GI involvement, and the subsequent scarcity of data in the literature, no consensus has emerged as to the optimal treatment of this condition. 8 For our patient, the paediatric oncologists decided to continue surveillance, due to the single site of disease relapse, making this lowrisk organ involvement, and because the resection margins of the resected rectal specimen were free of disease.…”