ABSTRACT
Purpose@#We aimed to determine the current application and survival trends of hematopoietic stem cell transplantation (HSCT) among Korean children and adolescents with cancer. @*Materials and Methods@#Data of patients aged < 20 years with KCD-10 (Korean Classifications of Diseases, 10th revision) C codes and specific designation codes were collected from the National Health Insurance Service database. Thirty claim codes for HSCT were included, and data from 2009 to 2019 were analyzed. @*Results@#The operational definition of pediatric cancer yielded an annual average of 2,000, with annual cases decreasing. In 2019, 221 HSCTs were performed, a decrease from the ten-year average of 276. Allografts outnumbered autografts with a ratio of 1.5:1. The source of allograft was bone marrow in 15% of patients in 2009; however, it substantially decreased to 3.3% in 2019. Furthermore, 70.5% of allogeneic HSCT used peripheral blood stem cell (PBSC) grafts, which increased to 89.3% by 2015. Cord blood utilization markedly decreased to 2.7% in 2018. The 5-year overall survival (OS) rate of all patients was 85.1%. Overall mortality decreased among patients who underwent recent HSCT, and they exhibited a higher 5-year OS rate. @*Conclusion@#In Korea, the number of pediatric patients with cancer is declining; however, the ratio of transplants to all patients remains constant. Patients who recently underwent transplantation showed better survival rates, possibly due to HSCT optimization. Korea showed a substantially greater PBSC utilization in pediatric HSCT. An in-depth examination encompassing donor relations and cause of death with a prospective registry is required in future studies.
ABSTRACT
Hepatocellular adenoma is an uncommon, benign liver tumor usually occurring in patients using estrogen or anabolic androgens and in those with a genetic disease, including glycogen storage disease. Hepatocellular adenomas can sometimes induce pain. However, it is usually asymptomatic. Moreover, few studies have reported cases of hepatocellular adenomas presenting with iron deficiency anemia. Herein, we report a pediatric case of a large hepatocellular adenoma, presenting with iron therapy-refractory iron deficiency anemia. A 14-year-old boy was diagnosed with hepatocellular adenoma during an anemia work-up. Improvement in iron deficiency anemia was observed after tumor resection.
ABSTRACT
22q11.2 deletion syndrome (22q11.2DS) is a disorder caused by microdeletions in the long arm of chromosome 22. It is one of the most common chromosomal microdeletion disorders. Clinical symptoms are caused by heterozygous deletion of chromosome 22q11.2 and include congenital heart diseases and palatal abnormalities.Bernard-Soulier syndrome (BSS) is a rare autosomal recessive bleeding disorder caused by deficiency of the glycoprotein Ib-IX-V complex (GPIb-IX-V), the receptor for von Willebrand factor. Glycoprotein Ib-beta (GPIbβ) gene mutation is one of the causes of BSS, and the GP1bβ gene is located on chromosome 22q11.2. Most 22q11DS patients do not have a bleeding issue since most of them have heterozygous deletion of the GPIbβ gene. However, we report a case in which a child with 22q11.2DS visited the hospital with subgaleal hemorrhage and was diagnosed with Bernard-Soulier syndrome with GPIbβ gene mutation by a platelet aggregation test and genetic sequencing.