Background: Hepatoblastoma is rare cancer that responds well to risk-based chemotherapy, and surgical treatment is needed to achieve complete remission and satisfactory survival rates in hepatoblastoma patients. In this study, we evaluated the clinical features and treatment outcomes of pediatric hepatoblastoma patients treated in our clinic. Methods: Eighteen patients with hepatoblastoma who were treated and followed up in our center between June 1999 and June 2020 were analyzed retrospectively. All patients were evaluated by a multidisciplinary team and managed using a risk-based protocol (SIOPEL-1 and SIOPEL-3). Results: The patients’ mean age at diagnosis was 38.33 ± 52.34 months. Sixteen patients (89%) received neoadjuvant chemotherapy, and 2 patients (11%) who underwent complete mass excision at diagnosis received adjuvant chemotherapy. After neoadjuvant therapy, the tumor was completely resected in 8 patients (45%), while liver transplantation was performed in 6 patients (34%) because complete resection of the tumor was not possible. Two patients died before surgical treatment. One patient relapsed with lung metastasis after salvage chemotherapy. She is alive without disease at 64 months. The mean follow-up time was 59.3 ± 49.8 months; 5-year overall and disease-free survival rates were 88.9% and 80.8%, respectively. The 5-year overall survival rate was 100% for both liver transplant and resected patients, whereas 5-year disease-free survival was lower in transplant patients (75% vs 100%, P < .001). Conclusion: Multidisciplinary follow-up is especially important for patients who may need liver transplantation. Some patients may benefit from new treatment options such as radiofrequency ablation and cyberknife treatment.
Background: Hematopoietic stem cell transplantation (HSCT) is a curative therapy option for hematologic malignancies. Iron overload is common in this patient group and can impact short-term and long-term nonrelapse mortality. Study Design: Retrospective observational cohort study. Aims: To evaluate the effect of iron load on early and late HSCT outcomes in patients with acute leukemia and myelodysplasia to assess the necessity of reducing iron load. Patients and Methods: Sixty patients who underwent HSCT in pediatric stem cell transplantation unit between 2000 and 2012 were evaluated retrospectively. The patients were divided into those with pretransplantation serum ferritin levels above and below the median value of 1299 ng/mL. Results: Forty-two (70%) of the patients were male, mean ages of the low and high ferritin groups were 85.43±9.42 and 118.56±10.04 months, respectively. Acute graft-versus-host disease (GVHD) within the first 100 days and acute liver GVHD were significantly more common in the high ferritin group (P<0.011 for both). Ferritin level was not associated with rates of engraftment syndrome, veno-occlusive disease, early/late infection, relapse, or overall and disease-free survival. Conclusions: In our study, significant result especially in terms of acute liver GVHD, was important to emphasize the need to be more careful in terms of acute liver GVHD risk in early liver pathologies in patients with high levels of ferritin after transplantation. In future large studies may be helpful to explain the relationship between acute liver GVHD and high ferritin levels.
Background: Proper clinical use of blood and blood products requires competent theoretical and practical knowledge of transfusion medicine. The Curriculum Development and Standard Determination System Medical Specialization Board is prepared Hematology Specialist Education Core Curriculum in Turkey. In this study, we aimed to determine the access of hematologists to the learning objectives defined by curriculum for the transfusion medicine and the factors affecting it.Methods: Hematologists who have been members of Turkish Hematology Society since 2013 have been included in the study, The survey questions were prepared based on the curriculum for transfusion medicine. The study was applied to hematologists with "survey monkey" application. The questionnaire consisted of a competence self-assessment with Likert scale and theoretical multiple-choice knowledge questions. Results: Of the 213 hematologists, 54 (25%) were included in the study. Hematologists rated their competences in the clinical competence areas as 3,65 ± 0,73 (median 3,60) as "I know but not t a sufficient level". The participants 'perception of competence was "I know, but not at a sufficient level'" with an average of 3.31 ± 0.84 (median3.5) in the blood banking field, while the average in hemapheresis and transfusion medicine was 4.04 ± 0.63 (median 4) as "enough". In interventional procedures, hematologists stated that their vocational competences were 2,79± 0,92 (median 2,93) on average as "I have an idea- I know, but not enough". The correct answer to 13 theoretical questions was an average of 6,96 ± 1,89 (median 7). Hematologists performing blood rotation felt significantly more competent than the physicians who could not do the rotation in the blood bank, blood banking t(52) = -3.9, p < .001 , transfusion medicine and interventional competence t(52) = -2.2, p = .04 . Physicians who believed that they are sufficient in the blood banking area, were more confident in transfusion medicine r(54) = .67, p <.001 and managing interventional procedures r(54) = .85, p <.001. Conclusion: In this study, hematologists generally felt more competent in subjects such as transfusion and therapeutic apheresis, which they often think of as not having enough knowledge in the area of blood banking. Hematologists have been more confident in the field of transfusion medicine as their years of expertise increased, but they did not feel better equipped in the fields of blood banking and interventional competence. The current results suggested that hematologists who are expected to be the blood bank supervisors do not internalize the area of blood banking, are not strong in their competence, and do not want to work in this area unless they are required.In hematology education curriculum, positive revisions in education can be achieved by revising blood banking curriculum and learning objectives, standardizing blood center rotations with content and duration, and support from online distance education programs.
Background: Data on the outcome and risk factors of pediatric patients with SARS-CoV-2 infection (COVID-19) following hematopoietic stem cell transplantation (HSCT) are limited. Objectives: We aimed to describe risk factors for a severe course and mortality. Method: In this nationwide study, data were collected retrospectively from 28 transplant centers. Results: One hundred ninety-six children [(63.8% male; median age 8.75 (IQR, 4.86-14.30)] who received allogeneic (n: 184, 93.9%) or autologous (n: 12, 6.1%) HSCT were included. The median time from HSCT to SARS-CoV-2 infection was 207.5 days (IQR, 110.2-207.5). The most common clinical manifestation was fever (58.2%), followed by cough (33.7%); 43 cases (21.9%) were asymptomatic. Lower respiratory tract disease (LRTD) and multisystem inflammatory syndrome in children (MIS-C) developed in 58 (29.6%) and 8 (4.1%) patients, respectively. Twenty-six patients (13.3%) required ICU admission. Nine patients died at a median of 17 days (min-max 1-33) after COVID-19 diagnosis, 6 of whom died due to the disease, with a COVID-19 lethality rate of 3.1%. The 6-week overall survival was 95.4% (95% CI 92.5-98.3). Multivariate analysis found that HSCT with a mismatched donor (OR, 8.98, p: 0.039) and LRTD (OR, 61.55, p: 0.001) were independent risk factors for ICU admission; MIS-C (OR, 9.55, p: 0.044) and lymphopenia (OR, 4.01, p: 0.030) at diagnosis were risk factors for mortality. Conclusion: Overall mortality was lower in children than in adult counterparts, and HSCT with a mismatched donor, lymphopenia, LRTD, MIS-C and ICU admission were important risk factors for adverse outcomes.
Background: Proper clinical use of blood products requires competent theoretical and practical knowledge of transfusion medicine. In this study, we aimed to evaluate levels of transfusion medicine knowledge and attainment of educational targets and identify factors affecting the education of transfusion medicine. Methods: A multicentric survey study was performed among final-year medical students. The questions were prepared based on learning objectives for transfusion medicine curriculum. The questionnaire focused on the safety of blood transfusion administration. Results: The survey included 727 (24%) of 3009 students enrolled in 13 medical schools. In the competence self-assessment, 65% of the students reported that transfusion medicine education was insufficient. Only 14% felt competent in recognizing transfusion complications and applying first-line treatment. For initiating and monitoring transfusions, 41% stated they could manage under supervision and 7% stated they had sufficient practice, while the remaining 52% considered themselves completely insufficient in this area. The 10 questions assessing basic knowledge and attainment of educational targets had 53 choices. This indicates that intern doctors were not able to recognize 50% of the correct and 30% of the incorrect information. Conclusion: A large proportion of medical students did not have adequate theoretical knowledge or self-assessed practical competency in transfusion medicine.
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