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Distinct Presentations of Langerhans Cell Histiocytosis in Children: A Case Series
Gashaw Arega1, Kirubel Asmelash1, Michael A Negussie2
1Department of Pediatrics and Child Health, College of Health Sciences, Addis Ababa University, Addis Ababa, Ethiopia.
Abstract:
Langerhans cell histiocytosis (LCH) is a rare pediatric histiocytic disorder characterized by diverse clinical manifestations, ranging from isolated lesions to severe multisystem involvement. This case series presents three distinct presentations observed in children. The first case involved a 4-year-old female presenting with generalized lymphadenopathy, polyuria, polydipsia, bilateral vision loss, and systemic symptoms, indicative of significant pituitary and multisystem involvement. Imaging revealed lesions involving the pituitary gland, hypothalamus, and sphenoid sinus. The second case described a 10-year-old male experiencing respiratory distress, significant weight loss, polyuria, and multiple lytic bone lesions. Diagnostic imaging identified extensive colonic involvement, bilateral hydronephrosis, and pulmonary lesions, emphasizing unusual systemic features. The third patient, an 18-month-old child, initially presented with persistent respiratory symptoms, a diffuse rash, severe acute malnutrition, and hepatomegaly and was initially misdiagnosed as having tuberculosis. Later imaging studies revealed extensive pulmonary cystic lesions. Immunohistochemistry from the tissue biopsy demonstrated CD1a positivity, confirming LCH diagnoses. Treatment strategies included standard induction protocols with vinblastine and corticosteroids. These cases show how LCH can present in many different ways in pediatric patients, often in unexpected patterns. Early recognition, thorough imaging, and histological confirmation are crucial for accurate diagnosis. Being aware of the wide range of symptoms can help ensure prompt treatment and better outcomes for this serious but manageable condition.
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