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The normal Langerhans cell and the LCH cell
Insights
Langerhans cells are key skin immune cells. Langerhans cell histiocytosis (LCH) involves abnormal LCH cells, prompting research into their origin and relationship to normal Langerhans cells.
Area of Science:
- Dermatology
- Immunology
- Pathology
Background:
- Epidermal Langerhans cells are bone marrow-derived, antigen-presenting dendritic cells.
- Characterized by Birbeck granules, they express MHC class II and CD1a, crucial for immune surveillance.
- Langerhans cells are vital in detecting skin-entry foreign antigens.
Purpose of the Study:
- To investigate the relationship between normal Langerhans cells and Langerhans cell histiocytosis (LCH) cells.
- To determine if LCH cells are arrested in development or a response to cellular insult.
- To understand the pathogenesis of LCH.
Main Methods:
- Phenotypic analysis of LCH cells.
- Comparison of LCH cell and normal Langerhans cell distribution.
- Ongoing studies to elucidate LCH cell ontogeny.
Main Results:
- LCH cells share a phenotype with early-stage activated Langerhans cells.
- LCH cells exhibit functional defects in antigen presentation.
- LCH disease distribution differs significantly from normal Langerhans cell distribution.
Conclusions:
- LCH cells appear to be Langerhans cells 'fixed' at an early activation stage.
- The distinct pathology suggests a complex relationship, not just a simple developmental arrest.
- Further research is critical to differentiate between developmental arrest and acquired defects in LCH pathogenesis.
Abstract:
The epidermal Langerhans cell is the bone marrow-derived dendritic, antigen-presenting cell of the skin. It is characterised by a unique intracytoplasmic organelle--the Birbeck granule--and constitutively expresses class II MHC molecules and the CD1a glycoprotein. The Langerhans cell represents one of the most potent antigen-presenting cells of the body, and fulfils an important role in detecting foreign antigen entering the body through the skin and in immune surveillance. The distribution of Langerhans cells is restricted to the skin, lymph nodes, bronchial mucosa and thymus. The discovery by Nézelof in 1973 that the lesional cells in the disease then called 'Histiocytosis X' contained Birbeck granules established the close relationship between the Langerhans cell and this disease and led ultimately to the adoption of the name Langerhans cell histiocytosis to replace the older term. The LCH cell expresses the phenotype of a Langerhans cell apparently 'fixed' at an early stage of cell activation. The LCH cell is, however, functionally defective in antigen presentation, and the tissue distribution of the disease--affecting bone, skin, lymph node, lung, liver, spleen, CNS, gastro-intestinal tract and bone marrow--is quite different from the normal distribution of the Langerhans cell. Studies are now under way throughout the world to investigate the relationship between the normal Langerhans cell and the LCH cell. Specifically we need to identify whether the LCH cell is a cell arrested at a specific time in normal Langerhans cell ontogeny or if it represents a response to a biological insult to the mature Langerhans cell or its precursors.
