Quality of life, fatigue, depression, attention deficits and pain after childhood Langerhans cell histiocytosis

1. Patients with LCH in childhood report suboptimal quality of life and high frequency of fatigue, depression, pain and attention deficits.

2. Patients with single system disease and those with multisystem disease with the longest treatment duration reported best quality of life.

Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia with variable clinical presentation, from self-healing single lesions to multisystem potentially fatal disease. Long-term consequences, including progressive central nervous system (CNS) neurodegeneration, are common. In this cross-sectional postal survey, we investigated how LCH affects long-term everyday life. All individuals ≥10 years diagnosed with LCH in childhood ≥5 years ago in Stockholm during 1990-2014 were invited to participate. Thirty-two of 61 eligible individuals (52%) answered questionnaires assessing health-related quality-of-life (HRQOL), fatigue, pain, depression, and attention deficits. Their median post-diagnosis time was 19.4 years. Overall, 14/32 (44%) had had multisystem disease, including four (12.5%) with risk organ involvement, and 17/32 (53%) had received systemic treatment. Five (16%) had CNS involvement, all with neurodegeneration. Mean total HRQOL score was 78.8 and mean total fatigue score 68.7 (Pediatric Quality of Life Inventory). Five (16%) reported a diagnosed neurodevelopmental disorder. In patients ≥15 years, 42% reported long-lasting pain and 27% had scores indicating depression. Poorer HRQOL correlated with fatigue and symptoms of depression and attention deficits. Patients with single system disease and patients with multisystem disease with the longest duration of systemic treatment reported best HRQOL. We conclude that patients with childhood LCH report high frequencies of fatigue, long-lasting pain, and symptoms of depression and attention deficit in the long-term, which are associated with poorer quality-of-life and should be evaluated at follow-up. We also raise the question if longer treatment may reduce long-term consequences and have a positive impact on perceived quality of life.