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Spontaneous healing of Langerhans cell histiocytosis (histiocytosis X)
L Corbeel1, E Eggermont, J Desmyter
1Department of Paediatrics, University of Leuven, Belgium.
Insights
This study reports a rare case of Langerhans cell histiocytosis in an infant, potentially linked to a novel virus. The infant showed significant improvement and resolution of symptoms over 14 months.
Area of Science:
- Pediatric Hematology/Oncology
- Virology
- Pathology
Background:
- Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder.
- Infantile LCH can present with multi-system involvement, including liver, spleen, and blood.
- Etiology of LCH remains unclear, with viral triggers being investigated.
Observation:
- A 3-month-old infant presented with severe anemia, thrombocytopenia, hepatosplenomegaly.
- Liver biopsy confirmed Langerhans cell histiocytosis (histiocytosis X).
- Patient's lymphocytes showed virus-like particles lacking reverse transcriptase activity.
Findings:
- The infant's condition improved significantly over 14 months.
- Hepatomegaly resolved, and hematological parameters normalized.
- Repeat liver biopsy showed reduced histiocytic infiltration, and viral cultures were negative.
Implications:
- This case suggests a potential viral association in some forms of infant Langerhans cell histiocytosis.
- The findings may warrant further investigation into non-retroviral viral particles as potential LCH triggers.
- Spontaneous resolution observed in this case offers insights into LCH pathogenesis and potential therapeutic avenues.
Abstract:
A 3-month-old male infant presented with pallor, hepatomegaly (4.5 cm), splenomegaly (1.5 cm), anaemia (Hb 6 g/dl) and thrombocytopenia (16 X 10(9)/l). A liver biopsy was diagnostic for Langerhans cell histiocytosis (histiocytosis X). The patient's lymphocytes, co-cultured with neonatal lymphocytes, were positive for virus-like particles without reverse transcriptase activity. The hepatomegaly diminished after 6 months and a second liver biopsy showed decreased histiocytic infiltration. A second viral blood culture remained negative. After 14 months, the hepatomegaly had disappeared completely and there were no more abnormal haematological or clinical findings.