Gyermekkori Langerhans-sejtes histiocytosissal szerzett magyarországi tapasztalataink

Translated title of the contribution: Experiences with Langerhans' cell histiocytosis in children in Hungary

J. Müller, Koós Rozália, M. Garami, P. Hauser, Borgulya Gábor, D. Schuler, Benyó Gábor, Magyarosy Edina, Galántai Ilona, Milei Krisztina, Török Katalin, Bárdi Edit, Hunyadi Katalin, Gábor Krisztina, Masáth Péter, L. Bognár

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

Background: Langerhans cell histiocytosis (LCH) in children is relatively rare, and the long-term analysis of therapy results has not been done yet in Hungary. Purpose: In this review we summarise the incidence, clinical features, prognostic risk factors and treatment results of children's LCH in Hungary, using data from the National Childhood Cancer Registry in Hungary in a 20-year period between 1981 and 2000. Results: From January 1981 to December 2000, 111 children under 18 years of age were newly diagnosed with LCH in Hungary. The male-female ratio was 1.36:1, the mean age: 4 years 11 months. The minimal and median follow-up time was 3.48 years and 10.98 years respectively. 38 children had singlesystem disease, while in 73 cases we found systemic dissemination already at the time of diagnosis. Twenty-two patients were treated only by local surgery, 7 by surgery with local irradiation and 5 children received only local irradiation. In two cases remission was obtained with local steroid administration. 75 patient received chemotherapy. During the twenty years 14 children died, 9 due to the progression of the disease. Sixteen of the 111 patients had relapse with a mean of 2.16±1.29 years after the first diagnosis. Three patients with relapse got chemotherapy generally used in lymphoma and remission was achieved. The overall survival of all patients (n=111) was 88.3±3.1% at 5 years and 87.3±3.2% at 10 and 20 years. Conclusion: Childhood LCH is a well treatable disease and the survival rate is high. Even disseminated diseases have a quite good prognosis in childhood.

Original languageHungarian
Pages (from-to)289-295
Number of pages7
JournalMagyar Onkologia
Volume48
Issue number4
Publication statusPublished - 2004

Fingerprint

Langerhans Cell Histiocytosis
Hungary
Recurrence
Drug Therapy
Registries
Disease Progression
Lymphoma
Survival Rate
Steroids
Survival
Incidence
Therapeutics
Neoplasms

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

Cite this

Gyermekkori Langerhans-sejtes histiocytosissal szerzett magyarországi tapasztalataink. / Müller, J.; Rozália, Koós; Garami, M.; Hauser, P.; Gábor, Borgulya; Schuler, D.; Gábor, Benyó; Edina, Magyarosy; Ilona, Galántai; Krisztina, Milei; Katalin, Török; Edit, Bárdi; Katalin, Hunyadi; Krisztina, Gábor; Péter, Masáth; Bognár, L.

In: Magyar Onkologia, Vol. 48, No. 4, 2004, p. 289-295.

Research output: Contribution to journalArticle

Müller, J, Rozália, K, Garami, M, Hauser, P, Gábor, B, Schuler, D, Gábor, B, Edina, M, Ilona, G, Krisztina, M, Katalin, T, Edit, B, Katalin, H, Krisztina, G, Péter, M & Bognár, L 2004, 'Gyermekkori Langerhans-sejtes histiocytosissal szerzett magyarországi tapasztalataink', Magyar Onkologia, vol. 48, no. 4, pp. 289-295.
Müller, J. ; Rozália, Koós ; Garami, M. ; Hauser, P. ; Gábor, Borgulya ; Schuler, D. ; Gábor, Benyó ; Edina, Magyarosy ; Ilona, Galántai ; Krisztina, Milei ; Katalin, Török ; Edit, Bárdi ; Katalin, Hunyadi ; Krisztina, Gábor ; Péter, Masáth ; Bognár, L. / Gyermekkori Langerhans-sejtes histiocytosissal szerzett magyarországi tapasztalataink. In: Magyar Onkologia. 2004 ; Vol. 48, No. 4. pp. 289-295.
@article{bb297de843f043acb28bc9a1d1e63ba6,
title = "Gyermekkori Langerhans-sejtes histiocytosissal szerzett magyarorsz{\'a}gi tapasztalataink",
abstract = "Background: Langerhans cell histiocytosis (LCH) in children is relatively rare, and the long-term analysis of therapy results has not been done yet in Hungary. Purpose: In this review we summarise the incidence, clinical features, prognostic risk factors and treatment results of children's LCH in Hungary, using data from the National Childhood Cancer Registry in Hungary in a 20-year period between 1981 and 2000. Results: From January 1981 to December 2000, 111 children under 18 years of age were newly diagnosed with LCH in Hungary. The male-female ratio was 1.36:1, the mean age: 4 years 11 months. The minimal and median follow-up time was 3.48 years and 10.98 years respectively. 38 children had singlesystem disease, while in 73 cases we found systemic dissemination already at the time of diagnosis. Twenty-two patients were treated only by local surgery, 7 by surgery with local irradiation and 5 children received only local irradiation. In two cases remission was obtained with local steroid administration. 75 patient received chemotherapy. During the twenty years 14 children died, 9 due to the progression of the disease. Sixteen of the 111 patients had relapse with a mean of 2.16±1.29 years after the first diagnosis. Three patients with relapse got chemotherapy generally used in lymphoma and remission was achieved. The overall survival of all patients (n=111) was 88.3±3.1{\%} at 5 years and 87.3±3.2{\%} at 10 and 20 years. Conclusion: Childhood LCH is a well treatable disease and the survival rate is high. Even disseminated diseases have a quite good prognosis in childhood.",
author = "J. M{\"u}ller and Ko{\'o}s Roz{\'a}lia and M. Garami and P. Hauser and Borgulya G{\'a}bor and D. Schuler and Beny{\'o} G{\'a}bor and Magyarosy Edina and Gal{\'a}ntai Ilona and Milei Krisztina and T{\"o}r{\"o}k Katalin and B{\'a}rdi Edit and Hunyadi Katalin and G{\'a}bor Krisztina and Mas{\'a}th P{\'e}ter and L. Bogn{\'a}r",
year = "2004",
language = "Hungarian",
volume = "48",
pages = "289--295",
journal = "Magyar Onkologia",
issn = "0025-0244",
publisher = "Akademiai Kiado",
number = "4",

}

TY - JOUR

T1 - Gyermekkori Langerhans-sejtes histiocytosissal szerzett magyarországi tapasztalataink

AU - Müller, J.

AU - Rozália, Koós

AU - Garami, M.

AU - Hauser, P.

AU - Gábor, Borgulya

AU - Schuler, D.

AU - Gábor, Benyó

AU - Edina, Magyarosy

AU - Ilona, Galántai

AU - Krisztina, Milei

AU - Katalin, Török

AU - Edit, Bárdi

AU - Katalin, Hunyadi

AU - Krisztina, Gábor

AU - Péter, Masáth

AU - Bognár, L.

PY - 2004

Y1 - 2004

N2 - Background: Langerhans cell histiocytosis (LCH) in children is relatively rare, and the long-term analysis of therapy results has not been done yet in Hungary. Purpose: In this review we summarise the incidence, clinical features, prognostic risk factors and treatment results of children's LCH in Hungary, using data from the National Childhood Cancer Registry in Hungary in a 20-year period between 1981 and 2000. Results: From January 1981 to December 2000, 111 children under 18 years of age were newly diagnosed with LCH in Hungary. The male-female ratio was 1.36:1, the mean age: 4 years 11 months. The minimal and median follow-up time was 3.48 years and 10.98 years respectively. 38 children had singlesystem disease, while in 73 cases we found systemic dissemination already at the time of diagnosis. Twenty-two patients were treated only by local surgery, 7 by surgery with local irradiation and 5 children received only local irradiation. In two cases remission was obtained with local steroid administration. 75 patient received chemotherapy. During the twenty years 14 children died, 9 due to the progression of the disease. Sixteen of the 111 patients had relapse with a mean of 2.16±1.29 years after the first diagnosis. Three patients with relapse got chemotherapy generally used in lymphoma and remission was achieved. The overall survival of all patients (n=111) was 88.3±3.1% at 5 years and 87.3±3.2% at 10 and 20 years. Conclusion: Childhood LCH is a well treatable disease and the survival rate is high. Even disseminated diseases have a quite good prognosis in childhood.

AB - Background: Langerhans cell histiocytosis (LCH) in children is relatively rare, and the long-term analysis of therapy results has not been done yet in Hungary. Purpose: In this review we summarise the incidence, clinical features, prognostic risk factors and treatment results of children's LCH in Hungary, using data from the National Childhood Cancer Registry in Hungary in a 20-year period between 1981 and 2000. Results: From January 1981 to December 2000, 111 children under 18 years of age were newly diagnosed with LCH in Hungary. The male-female ratio was 1.36:1, the mean age: 4 years 11 months. The minimal and median follow-up time was 3.48 years and 10.98 years respectively. 38 children had singlesystem disease, while in 73 cases we found systemic dissemination already at the time of diagnosis. Twenty-two patients were treated only by local surgery, 7 by surgery with local irradiation and 5 children received only local irradiation. In two cases remission was obtained with local steroid administration. 75 patient received chemotherapy. During the twenty years 14 children died, 9 due to the progression of the disease. Sixteen of the 111 patients had relapse with a mean of 2.16±1.29 years after the first diagnosis. Three patients with relapse got chemotherapy generally used in lymphoma and remission was achieved. The overall survival of all patients (n=111) was 88.3±3.1% at 5 years and 87.3±3.2% at 10 and 20 years. Conclusion: Childhood LCH is a well treatable disease and the survival rate is high. Even disseminated diseases have a quite good prognosis in childhood.

UR - http://www.scopus.com/inward/record.url?scp=15744395891&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=15744395891&partnerID=8YFLogxK

M3 - Article

C2 - 15655573

AN - SCOPUS:15744395891

VL - 48

SP - 289

EP - 295

JO - Magyar Onkologia

JF - Magyar Onkologia

SN - 0025-0244

IS - 4

ER -