title: Langerhans cell histiocytosis--clinicopathological reappraisal and human leucocyte antigen association.
abstract: We have examined the clinicopathological correlates of 74 patients with histologically confirmed Langerhans cell histiocytosis. Factors that influenced disease outcome included, three or more organ/systems being involved, a disease onset before the age of 2 years, the involvement of certain vital organs/systems such as liver/spleen, bone marrow and lungs, and male gender. The total number of involved organs/systems was the single most important determinant of disease outcome. Mortality rate in patients with three or more organs/systems involved, was 26%, as compared with 0% in the group with one or two organs/systems involved (chi 2 = 11.2, P = 0.008). There were no familial cases in our series, but we looked for a possible immunogenetic association by tissue typing 46 Caucasian sufferers and comparing the results with 117 controls. We used normal peripheral blood lymphocytes in 39 cases, Epstein-Barr virus-transformed lymphoblastoid cell lines in 12 cases, and both peripheral blood and Epstein-Barr virus-transformed lymphocytes in five cases. The HLA-B7 antigen was significantly increased Langerhans cell histiocytosis patients (19 of 46 = 41.3%) compared with 19 of 117 (16.2%) in the control group chi 2 = 11.2, relative risk = 3.6, P value after correction = 0.013). Attempt to stratify the disease into single-system or multisystem disease did not result in any significant association.
authors.affiliation: Unit of Dermatology, Royal Postgraduate Medical School, London, U.K.
authors.forename: R C
authors.initials: RC
authors.lastname: Yu
authors.forename: A C
authors.initials: AC
authors.lastname: Chu
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002472:Cell Transformation, Viral; D002648:Child; D002675:Child, Preschool; D005260:Female; D006680:HLA Antigens / Q000032:analysis*; D015793:HLA-B7 Antigen / Q000032:analysis; D006646:Histiocytosis, Langerhans-Cell / Q000276:immunology* / Q000473:pathology*; D006650:Histocompatibility Testing; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012737:Sex Factors
chemical_list: D006680:HLA Antigens; D015793:HLA-B7 Antigen