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.
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| http://dx.doi.org/10.1097/MS9.0000000000002607 | DOI Listing |
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Langerhans cell histiocytosis (LCH), juvenile xanthogranuloma (JXG) family lesions, and Rosai-Dorfman-Destombes disease (RDD) are now classified by the World Health Organization (WHO) under the heading of histiocytic/dendritic cell neoplasms. Each disease may manifest as a focal lesion, as multiple lesions, or as a widespread aggressive systemic disease with visceral organ involvement. Erdheim-Chester disease (ECD) is a rare systemic disease process of adults with limited cases in children.
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January 2025
Department of Pediatrics, West China Second University Hospital, Sichuan University, Sichuan, China.
Langerhans cell histiocytosis (LCH) is characterized genetically by diverse gene mutations of the mitogen-activated protein kinase signaling cascade. BRAFN486_T491delinsK mutation is a rare mutation that involves the β2-αC ring domain, causing activation of the mitogen-activated protein kinase pathway, and is predicted to be resistant to the chemotherapy and BRAFV600E inhibitor in adult LCH cases. Here, we report a childhood LCH case with this novel BRAF mutation and had a good response to conventional chemotherapy.
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Article Synopsis
- Langerhans cell histiocytosis (LCH) is a rare disorder marked by the abnormal buildup of dendritic histiocytes in tissues, often presenting as single- or multi-system diseases, particularly affecting adults.
- In a reported case, a 46-year-old woman experienced back pain, motor and sensory deficits, and was diagnosed with a compression fracture in the spine, alongside signs of central nervous system and salivary gland involvement.
- The patient underwent surgical intervention that included decompression and a biopsy, confirming the presence of LCH, highlighting the complexity and rarity of adult-onset LCH with extensive system involvement.
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Int J Surg Case Rep
January 2025
Skull Base Research Center, Loghman-Hakim Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran. Electronic address:
Introduction And Importance: Langerhans cell histiocytosis (LCH) is a rare disorder characterized by the proliferation of abnormal Langerhans cells, often presenting with symptoms that mimic common dermatological conditions such as hidradenitis suppurativa (HS). Accurate diagnosis is essential because LCH can affect multiple organ systems and necessitates distinct therapeutic approaches.
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