Treatment Results of Langerhans Cell Histiocytosis with LSH II Prot


Authors: H. Mottl;  M. Ganevová;  J. Radvanská;  M. Cháňová;  V. Šmelhaus;  R. Kodet 1;  M. Mrhalová 1;  M. Tichý 2
Authors‘ workplace: Klinika dětské hematologie a onkologie 2. LF UK a FNM, Praha ;  Ústav patologie a molekulární medicíny 2. LF UK a FNM, Praha 1;  Dětská neurochirurgie 2. LF UK a FNM, Praha 2
Published in: Čas. Lék. čes. 2005; 144: 753-755
Category: Original Article

Overview

Background.
The aim of study was to evaluate outcome of international treatment protocol LCH II for children with Langerhans cell histiocytosis treated in FN Motol.

Methods and Results.
Between November 1995 and December 2003, 46 children were treated, sex ratio M:F 29:17 and median age at diagnosis 6 years 8 months. 28 children (60.9%) suffered from monosystem disease with majority of bone lesions (23 times) with skull predominance (16 times). Surgery was primary treatment modality for monosystem disease. Five children with recurrence were successfully treated by protocol LCH II – LR (3x) and LCH III – LR /G2/, respectively. Eighteen children (39.1%) suffered from multisystem disease. 6 out of 18 patients were treated according to low-risk protocol LCH II – LR and 12 children by high-risk scheme LCH II – HR at the nonrandomized branch included etoposide. Recurrence was revealed in 11 patients and 10 of them reached 2nd or 3rd complete remission (CR) by 2 – chlorodeoxyadenosine (CDA) monotherapy, and 1 child reached 2nd CR by LCH II – HR scheme. Two children underwent irradiation after bone lesion excision as well as 1 child as supplemental treatment. Totally, 29 children (63.0 %) achieved 1st CR, 14 (30.4 %) 2nd CR, 2 (4.4 %) 3rd CR, and 1 child died because of LCH progression. There were no severe side effects of chemotherapy. Follow-up median time was 5 years 8 months (range 9 months – 9 years 6 months).

Conclusions.
LCH II protocol is safe and effective. Results revealed that treatment of patients with multisystem disease might demand some treatment modification.

Key words:
Langerhans cell histiocytosis, children, LCH II protocol, outcome.


Labels
Addictology Allergology and clinical immunology Angiology Audiology Clinical biochemistry Dermatology & STDs Paediatric gastroenterology Paediatric surgery Paediatric cardiology Paediatric neurology Paediatric ENT Paediatric psychiatry Paediatric rheumatology Diabetology Pharmacy Vascular surgery Pain management fenix.admin.empty
Login
Forgotten password

Don‘t have an account?  Create new account

Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.

Login

Don‘t have an account?  Create new account