The St Jude Total Therapy Study XV was the first clinical trial to prospectively use minimal residual disease levels during and after remission induction therapy to guide risk-directed treatment. We used the Total Therapy XV protocol with minimal modification in treating 115 newly diagnosed pediatric acute lymphoblastic leukemia patients from low- and middle-income groups from January 2011 to December 2017. The mean age at diagnosis was 5.97 ± 3.96 years. The median follow-up period was 88 months. Three (2.6%) patients had bone marrow relapse, and one (0.87%) had an isolated central nervous system relapse. Nineteen of the patients (16.52%) died due to infection-related complications, three (2.61%) died due to progressive disease, and one (0.87%) died due to hematopoietic stem cell transplant complications. Five-year overall survival was 80%, and event-free survival was 78.3%. Our results showed that the Total XV treatment protocol could be used successfully in patients with ALL from low- and middle-income populations. However, infection-related deaths remain a significant problem.
Objective: Neurofibromatosis type 1 (NF1) is the most common hereditary neurocutaneous syndrome. The most crucial morbidity of NF1 is tumors that may develop. Cases with café-au-lait macules (CALMs) which is the first clinical finding of NF1, due to the anxiety of its associated morbidity, are referred to the pediatric oncology clinic. In this study, we aimed to examine the characteristics of the patients who applied to our outpatient clinic with CALMs. Patients and Methods: The data of 157 pediatric patients who applied to our institution with the diagnosis of CALMs between June 2010 and November 2020 were analyzed retrospectively. Results: There were 157 pediatric cases referred to us for CALMs. According to the National Institutes of Health (NIH) diagnostic criteria, 109 (69.4%) cases were diagnosed with NF1. The diagnosis of 22 cases with NF1 were supported by genetic examination. Optic glioma was detected in 39 (24.8%) cases. In 15 (38.4%) of cases with optic glioma, visual functions were also affected. Second diagnostic criterion did not develop during the follow-up period, except for macules, in 48 cases (30.5%). Conclusion: In cases with multiple CALMs, the probability of NF1 diagnosis is high, and close and regular follow-up is of great importance in catching the development of the second clinical criterion and minimizing its morbidity.
Background When developed countries are considered; in the treatment of childhood acute lymphoblastic leukemia (ALL), the survival rate has reached 90% in recent years. We aimed to examine the survival rate and the factors that may affect this rate, especially in our patients treated with the ST Jude Total Therapy XV protocol. Procedures Pediatric patients aged 1-18 years, who were treated at our hospital and completed their treatment between January 2011 and December 2018, and only pre-B and T-cell leukemias treated with the St Jude Total Therapy XV protocol were included in the study. Results The 5-year event-free survival (EFS) and overall survival (OS) were 78.3% and 80%. We observed that some factors that affect survival, such as gender, blastic type, risk group, and number of WBCs, did not affect survival in our study. Even though it is known that the female gender has better EFS, in our study, the survival of girls was found to be lower than boys. Tragically, we observed that the most common cause of death (20/23, 87%) was infection and infection-related causes. Conclusions The primary purpose of the St Jude Total XV treatment protocol is to monitor minimal residual disease (MRD) and to guide treatment according to MRD results. It is also the removal of radiotherapy from treatment protocols by adding additional intra-thecal treatments (ITT). However, unfortunately, if we cannot protect our patients from infection and other related factors, the factors that make a difference in treatment become meaningless.