Nonneoplastic causes of death in neurofibromatosis 1: A cohort study with long-term follow-up.

Abstract

Neurofibromatosis 1 (NF1) is associated with reduced life expectancy due to malignancies; however, the risk of nonneoplastic causes of death is less well characterized. A total of 1622 persons with NF1, identified through either the Danish National Patient Registry or a clinical database, and 15,856 matched comparators born between 1951 to 2002 were followed from either age 20 or NF1 diagnosis. Death causes were from the Danish Cause of Death Register. Cumulative risks of different causes of death were compared between persons with and without NF1, and years of life lost (YLL) were calculated. Overall, 259 persons with NF1 (16.0%) and 578 comparators (3.6%) died during follow-up. The most frequent nonneoplastic causes of death in NF1 were diseases in the circulatory and nervous systems, with the latter showing a statistically significant higher 70-year cumulative risk in NF1. An excess of 6.1 (4.7-7.5) YLL within age 20 to 60 was seen in NF1 including 2.5 (1.5-3.5) years from nonneoplastic causes, and 3.6 (2.5-4.7) years from neoplasms. Persons with NF1 showed an increased risk of death because of nervous system diseases and neoplasms and a reduced life expectancy, with nonneoplastic causes accounting for approximately half of the YLL.