Patients treated with vincristine predictably develop peripheral neuropathy. The aim of our study was to investigate the pattern of vincristine-induced neuropathy in children by nerve conduction studies and somatosensory-evoked potentials (SSEPs). We included data from 39 children who received vincristine for various pediatric malignancies, and we performed initial and follow-up (after a minimum of 4 doses of vincristine 1.5 mg/m) conduction studies in 27 patients and SSEPs studies in 34 patients. On follow-up the most prevalent symptoms were paresthesias (44%) and constipation (22%), and the most common neurological sign was impaired myotatic reflexes (89%). Performing nerve conduction studies we found that significant reductions were measured for distal amplitudes, distal latencies were prolonged, and conduction velocities were relatively preserved. The most pronounced differences in amplitudes and distal latencies were measured in the peroneal nerves. Changes of SSEPs studies were subtle. Vincristine-induced neuropathy presents with primary axonal involvement and is more pronounced on motor neurons. We found a trend between higher age and higher dose and the degree of neuropathy in our group of patients.
Background IKZF1 gene deletions have been identified as a poor prognostic factor in pediatric B-cell acute lymphoblastic leukemia (B-ALL), especially in the presence of co-occurring deletions (IKZF1 plus profile). This study aimed to determine the frequency of IKZF1 deletions and deletions in other B-cell differentiation and cell cycle control genes, and their prognostic impact in Slovenian pediatric B-ALL patients. Patients and methods We studied a cohort of 99 patients diagnosed with B-ALL from January 2012 to December 2020 and treated according to the ALL IC-BFM 2009 protocol. Eighty-eight bone marrow or peripheral blood samples were analysed for copy number variations (CNVs) using the SALSA MLPA P335 ALL-IKZF1 probemix. Results At least one CNV was detected in more than 65% of analysed samples. The most frequently altered genes were PAX5 and CDKN2A/B (30.7%, 26.1%, and 25.0%, respectively). Deletions in IKZF1 were present in 18.2% of analysed samples and were associated with an inferior 5-year event-free survival (EFS; 54.8% vs. 85.9%, p = 0.016). The IKZF1 plus profile was identified in 12.5% of the analysed samples, and these patients had an inferior 5-year EFS than those with deletions in IKZF1 only and those without deletions (50.8% vs. 75.0% vs. 85.9%, respectively, p = 0.049). Overall survival (OS) was also worse in patients with the IKZF1 plus profile than those with deletions in IKZF1 only and those without deletions (5-year OS 76.2% vs. 100% vs. 93.0%, respectively). However, the difference between the groups was not statistically significant. Conclusions Our results are in concordance with the results obtained in larger cooperative clinical trials. Copy number variations analysis using the SALSA MLPA kit is a reliable tool for initial diagnostic approach in children with B-ALL, even in smaller institutions in low- and middle-income countries.
Necrotizing fasciitis is a potentially life-threatening infection of deep skin layers and subcutaneous tissues that can easily spread across the fascia plate and is usually the result of a combined infection with anaerobic and aerobic microorganisms. The patient typically complains of excruciating pain, which is not necessarily in accordance with clinical signs. Early recognition of the condition is very important, and aggressive treatment with a combination of antibiotics and surgical procedure is crucial. We present a case of a 15-year-old girl with acute lymphoblastic leukemia who developed necrotizing fasciitis after venous access port implantation during induction chemotherapy.
Treatment and long-term outcome in Slovenian children and adolescents, treated for acute lymphoblastic leukaemia (ALL) were evaluated, mainly considering diff erences between treatment regimens being evolved during time. From year 1967From year through 2004 were treated and six therapeutic schemes had been used. Retrospectively 5-year overall survival ± standard error (5yOS), relapse rate (RR) and second neoplasm (SN) were estimated according to treatment regimen, age, and where recorded, also according to BFM risk group, white blood cell count and disease subtype. For treatment regimens used after year 1992, 5-year eventfree survival ± standard error (5yEFS) was added to our observations. Among 75 pts enrolled in pre-POG treatment regimen, the probability of 5yOS was 25.3 ± 5.0%, while no data for RR and SN analysis were available. For 91 pts treated according to POG derived treatment, the probability of 5yOS was 61.6 ± 5.1%, with 64% RR and 5.5% occurrence of SN. Probability of 5yOS of 74.3 ± 7.4%, RR of 37% and 8.5% occurrence of SN were reached for 35 pts enrolled in ALL-BFM 83 protocol. ALL-BFM 86 protocol included 62 pts with 69.4 ± 5.9% probability of 5yOS, 33% RR and 3.2% occurrence of SN. For ALL-BFM 90 protocol the 5yEFS and 5yOS for 71 pts was 77.3 ± 5.2% and 81.8 ± 4.8%, respectively, with 17% RR and 1.5% occurrence of SN. Fifty-six patients enrolled in ALL-BFM 95 protocol reached 83.0 ± 5.2% 5yEFS and 92.6 ± 3.6% 5yOS, with 16% RR and 1.7% occurrence of SN. Stepwise rise of OS, decline in RR, reducing occurrence of SN and, for ALL-BFM 90 and ALL-BFM 95 protocol, improvements in EFS, were observed through time according to diff erent treatment regimens. General improvement regarding OS, RR and occurrence of SN was most obvious after the application of BFM regimen with evident continuous rise in OS and decline in RR and occurrence of SN thereafter.
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