A clinicopathological study of Langerhans cell histiocytosis.
BACKGROUND
Langerhans cell histiocytosis encompasses a wide clinical spectrum, from a benign localized disease to acute generalized disease with fatal outcome. Here the histological features and clinical data for thirteen patients are reviewed and compared. The antigenic phenotype of the proliferating cells in Langerhans cell histiocytosis was also studied.


METHODS
The antibodies used included S-100 protein, vimentin, peanut agglutinin, HLA-DR, leukocyte common antigen, UCHL-1, CD 20, lysozyme, alpha 1-antitrypsin and epithelial membrane antigen.


RESULTS
Six patients had localized lesions affecting a bone or its surrounding soft tissue. Four patients had multifocal disease without organ dysfunction. All patients of these two categories survived, except for one adult who died of hypertensive cerebral hemorrhage. Three patients had multifocal disease with liver, lung or bone marrow dysfunction. One died and two were lost to follow up.


CONCLUSIONS
There was no significant difference in pathological findings between localized disease and multifocal disease. Immunohistochemical examinations using a panel of antibodies, including S-100 protein, peanut agglutinin, HLA-DR and vimentin, were very helpful for diagnosis of Langerhans cell histiocytosis.
Department of Pathology, Mackay Memorial Hospital, Taipei, Taiwan, R.O.C.
B F
BF
Chen
D C
DC
Liang
D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D008297:Male; D009418:S100 Proteins / Q000032:analysis
D009418:S100 Proteins