Increased Mortality in LCH Driven By COPD & Other Cancers

Adults with Langerhans cell histiocytosis (LCH) have an increased risk for mortality from non-LCH causes compared with the general population, according to research published in Blood Advances. Ronald S. Go, MD, and colleagues examined long-term outcomes among 219 US adults with LCH. Patients with LCH experienced a significantly higher risk for overall mortality compared with adults without LCH (standardized mortality ratio [SMR], 2.66), especially for patients aged less than 55 at diagnosis (SMR, 5.94) and those with multisystem disease (SMR, 4.12). Dr. Go and colleagues identified second cancers in 16.4% of cases. LCH-associated deaths accounted for 36.1% of all mortality and occurred within 5 years of diagnosis. After 5 years, non-LCH causes of death, including second cancers, COPD, and cardiovascular diseases, were predominate. Cumulative, cause-specific mortality at 10 years was 7.8% for LCH, 3.9% for COPD, and 3.8% for secondary primary malignancies. “Our study highlights, to our knowledge, for the first time, that adults with LCH experience early and late mortality from non-LCH causes and the need for development of targeted survivorship programs to improve outcomes,” Dr. Go and colleagues wrote.