27 research outputs found
Gyermekkori Langerhans-sejtes histiocytosissal szerzett magyarországi tapasztalataink
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 single-system 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
The role of ABC-transporter gene polymorphisms in chemotherapy induced immunosuppression, a retrospective study in childhood acute lymphoblastic leukaemia
We examined the association of functional ABCB1 (MDR1) and ABCG2 (BCRP)
polymorphisms with acute side effects of chemotherapy. Analyses were performed on
clinical data from 138 patients treated with the ALL-BFM-95 protocol implying
several substrates of these transporters. ABCB1 3435T>C, 2677G>T/A 1236C>T and
ABCG2 421C>A genotypes were determined. A higher proportion of ABCB1 3435TT
patients suffered excessive infectious complications than those harbouring at
least one C allele (OR=2.5, p=0.03) during the whole half-year-long intensive
phase of chemotherapy. Weaker associations were calculated when ABCB1
1236T-2677T-3435T haplotype homozygotes were tested against the remaining part of
the population (OR=2.3, p=0.09). During the reinduction phase of therapy, the
occurrence of severe leukocytopenia was similar among ABCB1 genotype groups. The
frequency of any toxicities were not shown to differ according to the ABCG2
421C>A genotype. Our data suggest that the ABCB1 3435T>C genotype is associated
with the infectious complications of the applied chemotherapy regimen
Long-term survivors of childhood cancer: Cure and care. The Erice Statement
The number of individuals who have successfully completed treatment for a cancer diagnosed during childhood and are entering adulthood has been increasing. Members of the International Berlin-Frankfurt-Munster Early and Late Toxicity Educational Committee (ELTEC) invited 45 paediatric cancer experts - representing oncologists, psychologists, nurses, epidemiologists, parents, and survivors - from 13 European countries, with five additional experts from North America, to Erice, Sicily, on October 27-29, 2006, to discuss the circumstances in which the word "cure" should be used when speaking about children with cancer, and when and why continuing follow-up and care may be required. The objective of the gathering was to generate from the participants' personal and professional experiences an overview statement of the group's philosophy of cure and care of survivors of childhood cancer. The 10 points reflect what the group considers essential for the survivors' cure and car