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[Langerhans cell histiocytosis with isolated sternum involvement. A clinical case]
Lucila Di Nunzio1, Luján Gómez2, Aldana Rodríguez Gregori2
1Departamento de Urgencias, Hospital de Niños "Ricardo Gutiérrez" de Buenos Aires, Ciudad Autónoma de Buenos Aires. ludinunzio@gmail.com.
Insights
Langerhans Cell Histiocytosis (LCH) is a rare dendritic cell disorder. This case highlights LCH presenting as sternum pain in an 8-year-old, successfully treated with methylprednisolone.
Area of Science:
- Oncology
- Pediatrics
- Immunology
Background:
- Langerhans Cell Histiocytosis (LCH) is a rare clonal proliferative disorder of Langerhans cell precursors.
- LCH exhibits diverse clinical manifestations, from localized bone or skin lesions to severe multisystemic disease.
Observation:
- An 8-year-old girl presented with acute, severe sternum pain as the sole symptom.
- Imaging revealed a sternal osteolytic lesion, prompting further investigation.
Findings:
- Immunohistochemistry of fine needle aspiration confirmed LCH with CD1a positivity.
- The sternal lesion was diagnosed as Langerhans Cell Histiocytosis.
Implications:
- This case underscores the importance of considering LCH in pediatric sternal pain, even without systemic symptoms.
- Prompt diagnosis and treatment, such as with methylprednisolone, can lead to significant clinical improvement in LCH cases.
Abstract:
The Langerhans Cell Histiocytosis (LCH) is a rare condition, characterized by the proliferation of dendritic cells. Its clinical presentation is variable and ranges from an isolated skin or bone disease, mainly the skull, to a life-threatening multisystemic disease. This case is about a healthy 8-year-old girl with a history of four days of severe sternum pain and no other symptomatology. At the initial evaluation at the emergency department a chest X-ray (Figure 1) and thoracic computed tomography scan with 3-D reconstruction were performed and they showed the sternal osteolytic lesion (Figure 2). She was admitted to the hospital for further evaluation. The immunohistochemistry evaluation of the fine needle aspiration sample was positive for CD1a, confirming the diagnosis of histiocytosis. The patient received methylprednisolone 40 mg/ day with clinical improvement.
