Distinct Presentations of Langerhans Cell Histiocytosis in Children: A Case Series

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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Publication Details

Journal
Case Reports in Oncological Medicine
Published
2025-01-01
DOI
https://doi.org/10.1155/crom/5583430
Primary Topic
Histiocytic Disorders and Treatments
Type
article
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article

Distinct Presentations of Langerhans Cell Histiocytosis in Children: A Case Series

Michael A Negussie, Gashaw Arega, Abebe Mekonnen Woldeyohannes, Kirubel Asmelash
Case Reports in Oncological Medicine
Histiocytic Disorders and Treatments
article

Distinct Presentations of Langerhans Cell Histiocytosis in Children: A Case Series

Michael A Negussie, Gashaw Arega, Abebe Mekonnen Woldeyohannes, Kirubel Asmelash
article en

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.

Case Reports in Oncological MedicineVol. 2025(1)
Addis Ababa University (ET)
Openalex Percentile: Top 61%
Histiocytic Disorders and Treatments
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