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

Abstract: Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia with variable clinical presentation, ranging from self-healing single lesions to multisystem, potentially fatal disease. Long-term consequences, including progressive central nervous system (CNS) neurodegeneration, ar...

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Main Authors: Malin Sveijer, Désirée Gavhed, Helena Hertzberg, Eric Zander, Jan-Inge Henter, Tatiana von Bahr Greenwood
Format: Article
Language:English
Published: Elsevier 2025-08-01
Series:Blood Neoplasia
Online Access:http://www.sciencedirect.com/science/article/pii/S2950328025000330
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author Malin Sveijer
Désirée Gavhed
Helena Hertzberg
Eric Zander
Jan-Inge Henter
Tatiana von Bahr Greenwood
author_facet Malin Sveijer
Désirée Gavhed
Helena Hertzberg
Eric Zander
Jan-Inge Henter
Tatiana von Bahr Greenwood
author_sort Malin Sveijer
collection DOAJ
description Abstract: Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia with variable clinical presentation, ranging 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 aged ≥10 years diagnosed with LCH in childhood ≥5 years ago in Stockholm from 1990 to 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 postdiagnosis time was 19.4 years. Overall, 14 of 32 (44%) had had multisystem disease, including 4 (12%) with risk organ involvement, and 17 of 32 (53%) had received systemic treatment. Five (16%) had CNS involvement, all with neurodegeneration. The mean total HRQOL score was 78.8 and the mean total fatigue score 68.7 (Pediatric Quality of Life Inventory). Five (16%) reported a diagnosed neurodevelopmental disorder. In patients aged ≥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 the 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 of whether longer treatment may reduce long-term consequences and have a positive impact on perceived quality of life.
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spelling doaj-art-00b2d6ff636e4093b975fe22f5c4a8b62025-08-20T03:30:44ZengElsevierBlood Neoplasia2950-32802025-08-012310009810.1016/j.bneo.2025.100098Quality of life, fatigue, depression, attention deficits, and pain after childhood Langerhans cell histiocytosisMalin Sveijer0Désirée Gavhed1Helena Hertzberg2Eric Zander3Jan-Inge Henter4Tatiana von Bahr Greenwood5Childhood Cancer Research Unit, Department of Women’s and Children’s Health, Karolinska Institutet, Stockholm, Sweden; Department of Pediatrics, Eskilstuna Hospital, Eskilstuna, Sweden; Centre for Clinical Research Sörmland, Uppsala University, Uppsala, SwedenChildhood Cancer Research Unit, Department of Women’s and Children’s Health, Karolinska Institutet, Stockholm, Sweden; Division of Pediatric Oncology, Astrid Lindgren Children’s Hospital, Karolinska University Hospital, Stockholm, SwedenChildhood Cancer Research Unit, Department of Women’s and Children’s Health, Karolinska Institutet, Stockholm, Sweden; BB S:t Göran, Capio S:t Göran Hospital, Stockholm, SwedenCenter of Neurodevelopmental Disorders, Department of Women's and Children's Health, Centre for Psychiatry Research, Karolinska Institutet, Stockholm, SwedenChildhood Cancer Research Unit, Department of Women’s and Children’s Health, Karolinska Institutet, Stockholm, Sweden; Division of Pediatric Oncology, Astrid Lindgren Children’s Hospital, Karolinska University Hospital, Stockholm, Sweden; Jan-Inge Henter, Department of Women’s and Children’s Health, Karolinska Institutet, Tomtebodavägen 18A, SE-171 77 Stockholm, Sweden;Childhood Cancer Research Unit, Department of Women’s and Children’s Health, Karolinska Institutet, Stockholm, Sweden; Division of Pediatric Oncology, Astrid Lindgren Children’s Hospital, Karolinska University Hospital, Stockholm, Sweden; Correspondence: Tatiana von Bahr Greenwood, Department of Women’s and Children’s Health, Karolinska Institutet, Tomtebodavägen 18A, SE-171 77 Stockholm, Sweden;Abstract: Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia with variable clinical presentation, ranging 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 aged ≥10 years diagnosed with LCH in childhood ≥5 years ago in Stockholm from 1990 to 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 postdiagnosis time was 19.4 years. Overall, 14 of 32 (44%) had had multisystem disease, including 4 (12%) with risk organ involvement, and 17 of 32 (53%) had received systemic treatment. Five (16%) had CNS involvement, all with neurodegeneration. The mean total HRQOL score was 78.8 and the mean total fatigue score 68.7 (Pediatric Quality of Life Inventory). Five (16%) reported a diagnosed neurodevelopmental disorder. In patients aged ≥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 the 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 of whether longer treatment may reduce long-term consequences and have a positive impact on perceived quality of life.http://www.sciencedirect.com/science/article/pii/S2950328025000330
spellingShingle Malin Sveijer
Désirée Gavhed
Helena Hertzberg
Eric Zander
Jan-Inge Henter
Tatiana von Bahr Greenwood
Quality of life, fatigue, depression, attention deficits, and pain after childhood Langerhans cell histiocytosis
Blood Neoplasia
title Quality of life, fatigue, depression, attention deficits, and pain after childhood Langerhans cell histiocytosis
title_full Quality of life, fatigue, depression, attention deficits, and pain after childhood Langerhans cell histiocytosis
title_fullStr Quality of life, fatigue, depression, attention deficits, and pain after childhood Langerhans cell histiocytosis
title_full_unstemmed Quality of life, fatigue, depression, attention deficits, and pain after childhood Langerhans cell histiocytosis
title_short Quality of life, fatigue, depression, attention deficits, and pain after childhood Langerhans cell histiocytosis
title_sort quality of life fatigue depression attention deficits and pain after childhood langerhans cell histiocytosis
url http://www.sciencedirect.com/science/article/pii/S2950328025000330
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