Clinical characteristics and survival of children with Langerhans cell hystiocytosis
INTRODUCTION Langerhans cell histiocytosis is a rare disease in children, initial presentation is variable, clinical course, prognosis and survival are mostly unpredictable. OBJECTIVE To summarise clinical characteristics and treatment results in children with Langerhans cell histiocytosis. METHOD R...
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Serbian Medical Society
2008-01-01
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doaj-d4bb8d9117cd4b30b2b7b9650c67302a2021-01-02T12:02:35ZengSerbian Medical SocietySrpski Arhiv za Celokupno Lekarstvo0370-81792008-01-011369-1051451810.2298/SARH0810514KClinical characteristics and survival of children with Langerhans cell hystiocytosisKrstovski NadaJanić DraganaDokmanović LidijaBrdar RadivojINTRODUCTION Langerhans cell histiocytosis is a rare disease in children, initial presentation is variable, clinical course, prognosis and survival are mostly unpredictable. OBJECTIVE To summarise clinical characteristics and treatment results in children with Langerhans cell histiocytosis. METHOD Retrospectively there were analyzed patients with LCH diagnosed and treated at Hematology Department of University Children's Hospital in Belgrade from 1990 to 2006. Clinical presentation, therapy and survival according to Kaplan-Meier's statistical test was analysed. RESULTS 30 patients were treated, aged from 4 months to 14 years, mean 3.9 years, median 2.3 years, 18 (60%) males, 12 (40%) females. A single system disease was diagnosed in 16 (53%) patients, of whom 6 patients with multifocal bone disease. All patients were in complete remission averagely following162 and 82 months respectively. Multisystem disease was found in 14 (47%) patients. The lymph nodes and skin were more frequently involved organs than the central nervous system (diabetes insipidus), lung, liver and spleen. The number of involved organs ranged from 2 to 8, mean 4.2. Four patients died due to disease progression 3, 16, 36 and 66 months after diagnosis. Nine patents with multisystem disease were in remission with 117 months of follow-up. One patient was lost on follow-up. CONCLUSION The clinical course of patients with a single system disease is usually benign while a multisystem disease has to be aggressively treated with precise initial evaluation and staging before therapy. http://www.doiserbia.nb.rs/img/doi/0370-8179/2008/0370-81790810514K.pdfhistiocytosischildrensurvival |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Krstovski Nada Janić Dragana Dokmanović Lidija Brdar Radivoj |
spellingShingle |
Krstovski Nada Janić Dragana Dokmanović Lidija Brdar Radivoj Clinical characteristics and survival of children with Langerhans cell hystiocytosis Srpski Arhiv za Celokupno Lekarstvo histiocytosis children survival |
author_facet |
Krstovski Nada Janić Dragana Dokmanović Lidija Brdar Radivoj |
author_sort |
Krstovski Nada |
title |
Clinical characteristics and survival of children with Langerhans cell hystiocytosis |
title_short |
Clinical characteristics and survival of children with Langerhans cell hystiocytosis |
title_full |
Clinical characteristics and survival of children with Langerhans cell hystiocytosis |
title_fullStr |
Clinical characteristics and survival of children with Langerhans cell hystiocytosis |
title_full_unstemmed |
Clinical characteristics and survival of children with Langerhans cell hystiocytosis |
title_sort |
clinical characteristics and survival of children with langerhans cell hystiocytosis |
publisher |
Serbian Medical Society |
series |
Srpski Arhiv za Celokupno Lekarstvo |
issn |
0370-8179 |
publishDate |
2008-01-01 |
description |
INTRODUCTION Langerhans cell histiocytosis is a rare disease in children, initial presentation is variable, clinical course, prognosis and survival are mostly unpredictable. OBJECTIVE To summarise clinical characteristics and treatment results in children with Langerhans cell histiocytosis. METHOD Retrospectively there were analyzed patients with LCH diagnosed and treated at Hematology Department of University Children's Hospital in Belgrade from 1990 to 2006. Clinical presentation, therapy and survival according to Kaplan-Meier's statistical test was analysed. RESULTS 30 patients were treated, aged from 4 months to 14 years, mean 3.9 years, median 2.3 years, 18 (60%) males, 12 (40%) females. A single system disease was diagnosed in 16 (53%) patients, of whom 6 patients with multifocal bone disease. All patients were in complete remission averagely following162 and 82 months respectively. Multisystem disease was found in 14 (47%) patients. The lymph nodes and skin were more frequently involved organs than the central nervous system (diabetes insipidus), lung, liver and spleen. The number of involved organs ranged from 2 to 8, mean 4.2. Four patients died due to disease progression 3, 16, 36 and 66 months after diagnosis. Nine patents with multisystem disease were in remission with 117 months of follow-up. One patient was lost on follow-up. CONCLUSION The clinical course of patients with a single system disease is usually benign while a multisystem disease has to be aggressively treated with precise initial evaluation and staging before therapy. |
topic |
histiocytosis children survival |
url |
http://www.doiserbia.nb.rs/img/doi/0370-8179/2008/0370-81790810514K.pdf |
work_keys_str_mv |
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