Langerhans cell histiocytosis (LCH) is a rare disorder of histiocyte proliferation. Previous case studies suggest a higher prevalence of hematologic and solid malignancies among LCH patients, possibly due to treatment with tumorigenic agents such as etoposide. We report the first large, single-institution experience of adult LCH patients with additional malignancies to study the characteristics of these patients. We identified 132 consecutive patients >18 years of age with histologically confirmed LCH at our center between 1990 and 2015. Our cohort of adult LCH patients demonstrates an unusually high number of additional malignancies. Our study includes predominantly malignancies diagnosed preceding or concurrent with LCH, suggesting a cause of malignancy independent of LCH treatment. Further exploration of the biology of this rare disease may elucidate the mechanism of frequent additional malignancies. You can find the full article here.
7th RARE DISEASES SUMMER SCHOOL 2019