LANGERHANS CELL HISTIOCYTOSIS - CLINICOPATHOLOGICAL REAPPRAISAL AND HUMAN-LEUKOCYTE ANTIGEN ASSOCIATION

We have examined the clinicopathological correlates of 74 patients wit h histologically confirmed Langerhans cell histiocytosis. Factors that influenced disease outcome included, three or more organs/systems bei ng involved, a disease onset before the age of 2 years, the involvemen t of certain vital organs/systems such as liver/spleen, bone marrow an d lungs, and male gender. The total number of involved organs/systems was the single most important determinant of disease outcome. Mortalit y rate in patients with three or more organs/systems involved, was 26% , as compared with O% in the group with one or two organs/systems invo lved (chi(2) = 11.2, P = 0.008). There were no familial cases in our s eries, but we looked for a possible immunogenetic association by tissu e typing 46 Caucasian sufferers and comparing the results with 117 con trols. We used normal peripheral blood lymphocytes in 39 cases, Epstei n-Barr virus-transformed lymphoblastoid cell lines in 12 cases, and bo th peripheral blood and Epstein-Barr virus-transformed lymphocytes in five cases. The HLA-B7 antigen was significantly increased in Langerha ns 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 int o single-system or multisystem disease did not result in any significa nt association.