Document Detail

Functional defect in cells involved in Langerhans cell histiocytosis.
MedLine Citation:
PMID:  8534124     Owner:  NLM     Status:  MEDLINE    
The characteristic cell type involved in Langerhans cell histiocytosis, 'LCH cells', express most of the enzyme histochemical and immunocytochemical markers of normal epidermal Langerhans cells. It is not known, however, whether these LCH cells express the functional characteristics of normal epidermal Langerhans cells. We studied the alloantigen-presenting activity of LCH cells derived from lesional sites of three patients with the disease. Lesional cells expressing the CD1a molecule were enriched using either fluorescein-activated cell sorting or negative selection with indirect immunomagnetic beads, and functional activity was assessed using the 6-day primary allogeneic mixed-cell reaction. Compared to epidermal Langerhans cells from healthy controls, LCH cells showed minimal alloantigen-presenting activity on a per-cell basis. The diminished activity was not reversed by exogenous prostaglandin synthetase inhibitor or recombinant human IL-1 beta. This study confirms our previous report of a child, with fatal multisystem Langerhans cell histiocytosis suggesting that this disease represents a condition in which functionally defective cells of Langerhans cell phenotype accumulate and/or proliferate in various tissues. We postulate that the functional defect is a primary defect of these LCH cells that have acquired an as-yet-undetermined biological insult(s).
R C Yu; M Alaibac; A C Chu
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Archives of dermatological research     Volume:  287     ISSN:  0340-3696     ISO Abbreviation:  Arch. Dermatol. Res.     Publication Date:  1995  
Date Detail:
Created Date:  1996-01-30     Completed Date:  1996-01-30     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8000462     Medline TA:  Arch Dermatol Res     Country:  GERMANY    
Other Details:
Languages:  eng     Pagination:  627-31     Citation Subset:  IM    
Unit of Dermatology, Royal Postgraduate Medical School, Hammersmith Hospital, London, UK.
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MeSH Terms
Child, Preschool
Histiocytosis, Langerhans-Cell / immunology*,  metabolism
Immunomagnetic Separation
Indomethacin / pharmacology
Interleukin-1 / pharmacology
Isoantigens / immunology*
Prostaglandin-Endoperoxide Synthases / drug effects
Reg. No./Substance:
0/Interleukin-1; 0/Isoantigens; 53-86-1/Indomethacin; EC Synthases

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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