Few monozygotic twin pairs with concordant leukemia has been described so far. The interval between the onsets of overt disease in each of the twins is variable usually from some months to some years. We present a unique case of monozygotic and monohorionic twins who developed AML with unusually long difference of disease onset, in which we investigated the genetic background. Both twins carried a germline N-terminal frameshift CEBPA mutation, a 19-base pair deletion (c.147_165del, p.Glu50fs). Interestingly, at the time of diagnosis of AML both twins carried an additional identical somatic C-terminal mutation: an inframe insertion of aminoacid lysine (c.936_937dupAAG, p.313_314insLys). Twin A, also had the third mutation in C-terminal part of CEBPA gene, somatic inframe deletion of 50 aminoacids (c.911_1060del, p.304-353del). We suspect that this mutation has arisen in one twin and has been interplacentaly transferred to other twin in utero. The syngenic bone marrow donor twin developed AML 13 years after the diagnosis of her twin.
COBISS.SI-ID: 864940
Chemotherapy-induced oral mucositis (OM) is a debilitating side effect. In addition to standard therapy, patients often use complementary and alternative medicine to treat OM. Double blind randomised placebo controlled study assessing propolis (bee glue) efficacy for chemotherapy-induced severe OM treatment. Paediatric patients undergoing chemotherapy were randomly assigned to propolis (n=19) or placebo groups (n=21). Patients were introduced to a unified oral care protocol and asked to apply propolis or placebo to vestibular mucosa twice daily. Oral mucosa was assessed with the Oral Assessment Guide (OAG) twice a week when the patients were in hospital. Patients were followed for the period of the chemotherapy or for the first 6 months of the chemotherapy. An OAG score of 3 was considered to be severe OM and analysed.Main outcome measurementsThree dependent variables (a) OM episode frequency, (b) mean number of assessment visits, at which an OAG 3 score was noted, expressing mean OM duration, (c) mean number of OAG 3 scores expressing mean OM severity) were reduced to a single variable using principal component analysis. A new variable (FDS) was used as the dependent variable in ANCOVA model analysis to show the differences between study groups.ResultsSevere OM was seen in 42% and 48% of patients in the propolis and placebo group, respectively. FDS was not statistically significant between study groups (p=0.59).ConclusionsAccording to our study results, propolis cannot be recommended for severe OM treatment.
COBISS.SI-ID: 30911449
From 2002 to 2007, the International Berlin-Frankfurt-Münster Study Group conducted a prospective randomized clinical trial (ALL IC-BFM 2002) for the management of childhood acute lymphoblastic leukemia (ALL) in 15 countries on three continents. The aim of this trial was to explore the impact of differential delayed intensification (DI) on outcome in all risk groups.For this trial, 5,060 eligible patients were divided into three risk groups according to age, WBC, early treatment response, and unfavorable genetic aberrations. DI was randomized as follows: standard risk (SR), two 4-week intensive elements (protocol III) versus one 7-week protocol II; intermediate risk (IR), protocol III × 3 versus protocol II × 1; high risk (HR), protocol III × 3 versus either protocol II × 2 (Associazione Italiana Ematologia Oncologia Pediatrica [AIEOP] option), or 3 HR blocks plus single protocol II (Berlin-Frankfurt-Münster [BFM] option).At 5 years, the probabilities of event-free survival and survival were 74% (± 1%) and 82% (± 1%) for all 5,060 eligible patients, 81% and 90% for the SR (n = 1,564), 75% and 83% for the IR (n = 2,650), and 55% and 62% for the HR (n = 846) groups, respectively. No improvement was accomplished by more intense and/or prolonged DI. The ALL IC-BFM 2002 trial is a good example of international collaboration in pediatric oncology. A wide platform of countries able to run randomized studies in ALL has been established. Although the alternative DI did not improve outcome compared with standard treatment and the overall results are worse than those achieved by longer established leukemia groups, the national results have generally improved.
COBISS.SI-ID: 1232812