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Langerhans Cell Histiocytosis in an Infant Mimicking a Lymphoma at Presentation
Anjan Madasu1, Asim Noor Rana1, Saleh Banat1
1Pediatric Hematology and Oncology Unit, Dubai Hospital, Dubai, UAE.
Insights
Langerhans cell histiocytosis (LCH) is a rare infant disorder. This case highlights LCH presenting with respiratory distress, mediastinal mass, lymphadenopathy, and hepatosplenomegaly, emphasizing its importance in differential diagnosis.
Area of Science:
- Pediatric Oncology
- Hematology
- Immunology
Background:
- Langerhans cell histiocytosis (LCH) is a rare clonal dendritic cell proliferation.
- LCH presents with diverse clinical manifestations and unpredictable outcomes.
Purpose of the Study:
- To report a rare case of LCH in an infant with a complex presentation.
- To emphasize the importance of considering LCH in the differential diagnosis of infants with specific symptoms.
Main Methods:
- Case report of a 5-month-old infant.
- Clinical presentation analysis including respiratory distress, mediastinal mass, lymphadenopathy, and hepatosplenomegaly.
- Diagnostic confirmation via biopsy after initial empirical therapy for suspected lymphoma.
Main Results:
- The infant presented with severe respiratory distress, a large mediastinal mass, generalized lymphadenopathy, and hepatosplenomegaly.
- Initial suspicion of lymphoma was not confirmed; biopsy revealed LCH.
- Prompt diagnosis and appropriate therapy led to improved survival.
Conclusions:
- LCH can present with a rare combination of generalized lymphadenopathy, mediastinal mass, hepatosplenomegaly, and fever in infants.
- LCH should be included in the differential diagnosis for infants presenting with these combined symptoms.
- Timely biopsy is crucial for accurate diagnosis and management of suspected LCH.
Abstract:
Langerhans cell histiocytosis (LCH) is a rare disorder characterized by proliferation and accumulation of clonal dendritic cells with varied clinical presentation and an unpredictable course. We report a 5-month-old infant with LCH who presented with severe respiratory distress, a large mediastinal mass, significant generalized lymphadenopathy, and hepatosplenomegaly. Lymphoma, especially T cell lymphoblastic lymphoma, can present with superior mediastinal syndrome needing urgent empirical therapy without biopsy. However, lack of response prompted a biopsy which confirmed it to be a case of LCH and that leads to appropriate therapy and survival. There have been reports of LCH presenting with isolated mediastinal mass or with generalized lymphadenopathy, but the combined presentation of generalized lymphadenopathy with large mediastinal mass, hepatosplenomegaly, and fever in an infant has rarely been reported. Conclusion. LCH should also be considered in the differential diagnosis of an infant presenting with generalized lymphadenopathy, mediastinal mass, hepatosplenomegaly, and fever.
