Purpose: Chronic myeloid leukemia (CML) in children, adolescents, and young adults is rare and differs from older adults. This study evaluated the outcomes of allogeneic hematopoietic stem cell transplantation (HSCT) in young Korean CML patients during the tyrosine kinase inhibitor (TKI) era. Materials and Methods: A retrospective analysis of 35 CML patients aged < 40 years who underwent allogeneic HSCT from 2009 to 2019 was conducted using Korean Blood and Marrow Transplantation Registry data. Patients were grouped by age < 20 years at HSCT (group 1, n=15) and 20-40 years at HSCT (group 2, n=20). Survival outcomes including overall survival (OS), relapse-free survival (RFS), and event-free survival (EFS) were analyzed using the Kaplan-Meier method. Results: The median time between diagnosis and HSCT was 8.9 months. All the patients achieved engraftment but platelet recovery was significantly slower in group 1 (p=0.034). Acute and chronic graft-versus-host disease occurred in 54.3% and 34.3%, respectively. Five-year OS, RFS, and EFS rates of total patients were 66.8%, 50.8%, and 47.6%, with better OS was observed in group 1 by multivariable analysis (p=0.048). Disease status at HSCT was a significant predictor of OS (p=0.028), RFS (p=0.003), and EFS (p=0.004). Disease progression occurred in 13 out of 35 patients (37.1%); treatment-related mortality accounted for 63.6% of deaths (7 out of 11). Conclusion When performed at a younger age, allogeneic HSCT result in superior outcome in CML. Achieving remission before HSCT is critical for improved outcomes, highlighting the importance of pretransplant remission via optimal TKI strategies and minimal residual disease monitoring.

The increasing detection of papillary thyroid microcarcinoma (PTMC) has raised concerns about overtreatment.For low-risk PTMC, either immediate surgery or active surveillance (AS) can be considered. To support AS implementation, the Korean Thyroid Association convened a multidisciplinary panel and developed the first Korean guideline. AS is recommended to adults with pathologically proven Bethesda V-VI PTMC without clinical evidence of lymph node or distant metastasis, gross extrathyroidal extension, tracheal or recurrent laryngeal nerve invasion, or aggressive histology. Baseline assessment requires high‑resolution cervical ultrasound by experienced operators to rule out extrathyroidal extension, tracheal or recurrent laryngeal nerve invasion, and lymph node metastasis;contrast‑enhanced neck computed tomography is optional. Patient characteristics such as age, comorbidities, and capacity for long-term follow-up should be assessed. Shared decision-making should weigh the benefits and risks of surgery and AS, expected oncologic outcomes, complications, quality of life, anxiety, medical cost, and patient preference. Follow-up includes cervical ultrasound and thyroid function test every six months for two years, then annually. Disease progression, defined as significant tumor growth or newly detected nodal or distant metastasis, warrants surgery. Despite remaining uncertainties, this guideline offers a framework to ensure oncologic safety and support patient-centered active surveillance.

Background: Although many studies have been conducted on the association between the intercondylar notch size and the risk of anterior cruciate ligament (ACL) injury, few studies have examined its relationship with the condition after surgical treatment.Therefore, this study aimed to investigate the surgical outcomes of anatomical single-bundle ACL reconstruction according to intercondylar notch volumes. Methods: Medical records of patients who underwent anatomical single-bundle ACL reconstruction using a tibialis anterior allograft between 2015 and 2019 were retrospectively reviewed. For each sex, eligible patients were classified into two groups based on their percentile of intercondylar notch volumes, which were measured using postoperative three-dimensional computed tomography images (group S, ≤ 50th percentile of included patients; group L, > 50th percentile of included patients). Additional grouping was performed based on the group’s percentiles of normalized values of intercondylar notch volumes to body heights.Between-group comparative analyses were performed on the perioperative data and surgical outcomes in both objective and subjective aspects. Results: One hundred patients were included in the study. For male patients, there were no differences in the overall surgical outcomes between groups, whereas group L showed a significantly greater knee anteroposterior (AP) laxity than group S at the final follow-up (p = 0.042 for the side-to-side differences [SSD] at the maximum manual force). Similarly, there were no differences in the female patients in the overall surgical results between the groups, whereas group L showed a significantly greater knee AP laxity at the final follow-up (p = 0.020 for the SSD at 134 N; p = 0.011 for the SSD at the maximum manual force). Additional analyses based on the normalized values of the intercondylar notch volume showed consistent results for male patients, and additional grouping for female patients was identical to the existing grouping. Conclusions The surgical outcomes of anatomical single-bundle ACL reconstruction in patients with relatively small intercondylar notch volumes were comparable to those with large notch volumes, but rather showed favorable outcomes in postoperative knee AP laxity.

Background/Aims: The prognostic significance of 18F-fluorodeoxyglucose (FDG)-positron emission tomography-computed tomography (PET/CT) in peripheral T-cell lymphomas (PTCLs) are controversial. We explored the prognostic impact of sequential 18F-FDG PET/CT during frontline chemotherapy of patients with PTCLs. Methods: In total, 143 patients with newly diagnosed PTCLs were included. Sequential 18F-FDG PET/CTs were performed at the time of diagnosis, during chemotherapy, and at the end of chemotherapy. The baseline total metabolic tumor volume (TMTV) was calculated using the the standard uptake value with a threshold method of 2.5. Results: A baseline TMTV of 457.0 cm3 was used to categorize patients into high and low TMTV groups. Patients with a requirehigh TMTV had shorter progression-free survival (PFS) and overall survival (OS) than those with a low TMTV (PFS, 9.8 vs. 26.5 mo, p = 0.043; OS, 18.9 vs. 71.2 mo, p = 0.004). The interim 18F-FDG PET/CT response score was recorded as 1, 2–3, and 4–5 according to the Deauville criteria. The PFS and OS showed significant differences according to the interim 18F-FDG PET/CT response score (PFS, 120.7 vs. 34.1 vs. 5.1 mo, p < 0.001; OS, not reached vs. 61.1 mo vs. 12.1 mo, p < 0.001). Conclusions The interim PET/CT response based on visual assessment predicts disease progression and survival outcome in PTCLs. A high baseline TMTV is associated with a poor response to anthracycline-based chemotherapy in PTCLs. However, TMTV was not an independent predictor for PFS in the multivariate analysis.

Objective: The purpose of this study is to investigate the association of the apolipoprotein E (APOE) e4 genotype with cognition, brain volume, glucose metabolism, and amyloid deposition in patients with Alzheimer disease (AD). Methods: This is cross-sectional study of 69 subjects with AD. All subjects were divided into carriers and non-carriers of the e4 allele. Forty APOE e4 carriers and 29 APOE e4 non-carriers underwent neuropsychological, structural magnetic resonance imaging, [18F]fluorodeoxyglucose positron emission tomography scans (PET) and [18F]florbetaben amyloid PET. Analysis of co-variance was conducted to compare the differences on cognition, brain volume, glucose metabolism and amyloid deposition between APOE e4 carriers and non-carriers after controlling demographics. Results: APOE e4 carriers had 50% lower scores of Seoul Verbal Learning Test (delayed recall) compared to non-carriers (0.88±1.65 vs. 1.76±1.75, p<0.05). However, APOE e4 carriers performed better on other cognitive tests than non-carriers (Korean version of Boston Naming Test [11.04±2.55 vs. 9.66±2.82, p<0.05], Rey Complex Figure Test [25.73±8.56 vs. 20.15±10.82, p<0.05], and Stroop test [color response] [48.28±26.33 vs. 31.56±27.03, p<0.05]). APOE e4 carriers had slightly smaller hippocampal volume than non-carriers (3.09±0.38 vs. 3.32±0.38, p<0.05), but greater total brain cortical thickness (1.45±1.55 vs. 1.37±1.24, p<0.05). Amyloid deposition did not differ significantly between APOE e4 carriers and non-carriers, and no signifi-cant difference in glucose metabolism was found between groups. Conclusion We found that APOE e4 genotype is associated with cognition, brain volume in AD, suggesting that APOE e4 genotype could play an important role in the underlying pathogenesis of AD.

Background/Aims: We performed a prospective study to determine the efficacy and safety of rituximab including chemotherapy in CD20-positive acute lymphoblastic leukemia (ALL). Methods: Patients with newly diagnosed ALL, aged ≥ 15 years, were eligible for the study if their leukemic blast cells in bone marrow expressed CD20 ≥ 20% at the time of diagnosis. Patients received multiagent chemotherapy with rituximab. After achieving complete remission (CR), patients received five cycles of consolidation with concomitant rituximab. Rituximab was administered monthly from day 90 of transplantation for patients who received allogeneic hematopoietic cell transplantation. Results: In patients with Philadelphia (Ph)-negative ALL, 39 of 41 achieved CR (95.1%), the 2- and 4-year relapse-free survival (RFS) rates were 50.4% and 35.7%, and the 2- and 4-year overall survival (OS) rates were 51.5% and 43.2%, respectively. In the group with Ph-positive ALL, all 32 patients achieved CR, the 2- and 4-year RFS rates were 60.7% and 52.1%, and the 2- and 4-year OS rates were 73.3% and 52.3%, respectively. In the Ph-negative ALL group, patients with higher CD20 positivity experienced more favorable RFS (p < 0.001) and OS (p = 0.06) than those with lower CD20 positivity. Patients who received ≥ 2 cycles of rituximab after transplantation had significantly improved RFS (hazard ratio [HR], 0.31; p = 0.049) and OS (HR, 0.29; p = 0.021) compared with those who received < 2 cycles. Conclusions The addition of rituximab to conventional chemotherapy for CD20-positive ALL is effective and tolerable (Clinicaltrials. gov NCT01429610).

We report a patient with severe neurological deterioration due to leptomeningeal metastases involving brain and spinal cord from anaplastic lymphoma kinase (ALK)-positive lung adenocarcinoma, managed rapidly and successfully with lorlatinib therapy. A 48-year-old male patient presented with acute mental deterioration, severe headache, and weakness of both legs. The patient’s previous medical history included cerebral metastases from ALK-positive lung adenocarcinoma, which had been successfully managed via whole brain radiation therapy and gamma knife radiosurgery one year and three months before, respectively. Physical examination revealed neck stiffness and paraparesis with motor grade I.Gadolinium-enhanced brain MRI showed newly developed leptomeningeal enhancement along cerebellar folia, and whole spine MRI revealed similar leptomeningeal metastasis along the whole spinal axis. Lorlatinib was started orally with a dose of 100 mg/day. The patient showed rapid clinical improvement after one week. The patient was alert and the headache disappeared, while the paraparesis improved to normal ambulatory status. Two months of lorlatinib treatment resulted in almost complete disappearance of previous leptomeningeal enhancement of brain and spinal cord, and absence of newly developed metastatic lesions in the central nervous system, based on MRI results. The patient had been regularly followed with ongoing lorlatinib therapy for 5 months without any systemic complications or neurological abnormality. Conclusively, lorlatinib could be a rapid and effective treatment for patients with central nervous system leptomeningeal metastases arising from ALK-positive lung cancer.

BACKGROUND: Chronic cough is defined as a cough lasting more than 8 weeks and socio-economic burden of chronic cough is enormous. The characteristics of chronic cough in Korea are not well understood. The Korean Academy of Tuberculosis and Respiratory Diseases (KATRD) published guidelines on cough management in 2014. The current study evaluated the clinical characteristics of chronic cough in Korea and the efficacy of the KATRD guidelines. METHODS: This was a multi-center, retrospective observational study conducted in Korea. The participants were over 18 years of age. They had coughs lasting more than 8 weeks. Subjects with current pulmonary diseases, smokers, ex-smokers with more than 10 pack-years or who quit within the past 1 year, pregnant women, and users of cough-inducing medications were excluded. Evaluation and management of cough followed the KATRD cough-management guidelines. RESULTS: Participants with chronic cough in Korea showed age in the late forties and cough duration of more than 1 year. Upper airway cough syndrome was the most common cause of cough, followed by cough-variant asthma (CVA). Gastro-esophageal reflux diseases and eosinophilic bronchitis were less frequently observed. Following the KATRD cough-management guidelines, 91.2% of the subjects improved after 4 weeks of treatment. Responders were younger, had a longer duration of cough, and an initial impression of CVA. In univariate and multivariate analyses, an initial impression of CVA was the only factor related to better treatment response. CONCLUSION The causes of chronic cough in Korea differed from those reported in other countries. The current Korean guidelines proved efficient for treating Korean patients with chronic cough.

BACKGROUND: Chronic cough is defined as a cough lasting more than 8 weeks and socio-economic burden of chronic cough is enormous. The characteristics of chronic cough in Korea are not well understood. The Korean Academy of Tuberculosis and Respiratory Diseases (KATRD) published guidelines on cough management in 2014. The current study evaluated the clinical characteristics of chronic cough in Korea and the efficacy of the KATRD guidelines. METHODS: This was a multi-center, retrospective observational study conducted in Korea. The participants were over 18 years of age. They had coughs lasting more than 8 weeks. Subjects with current pulmonary diseases, smokers, ex-smokers with more than 10 pack-years or who quit within the past 1 year, pregnant women, and users of cough-inducing medications were excluded. Evaluation and management of cough followed the KATRD cough-management guidelines. RESULTS: Participants with chronic cough in Korea showed age in the late forties and cough duration of more than 1 year. Upper airway cough syndrome was the most common cause of cough, followed by cough-variant asthma (CVA). Gastro-esophageal reflux diseases and eosinophilic bronchitis were less frequently observed. Following the KATRD cough-management guidelines, 91.2% of the subjects improved after 4 weeks of treatment. Responders were younger, had a longer duration of cough, and an initial impression of CVA. In univariate and multivariate analyses, an initial impression of CVA was the only factor related to better treatment response. CONCLUSION The causes of chronic cough in Korea differed from those reported in other countries. The current Korean guidelines proved efficient for treating Korean patients with chronic cough.

Objectives: ：It is found that imbalance in activity-dependent neuroprotective protein(ADNP) and the homologous protein ADNP2 in schizophrenia may impact the disease progression. Yet, further research is required to clarify their connection to schizophrenia. This is a pilot study for a family-based association analysis of ADNP2 gene in a Korean population with schizophrenia. Methods: ：Twenty-seven probands with schizophrenia were recruited with their parents and siblings. We have used lifetime dimensions of psychosis scale for measuring psychotic features. Promising endophenotypic markers such as age at interview, apparent onset, apparent onset of psychosis, and first treatment age were also included. We analyzed 2 single nucleotide polymorphisms (SNPs) of ADNP gene. Then, we performed family based association test(FBAT) and linkage disequilibrium analyses for each individual SNPs. Results: ：A significant SNP of ADNP2 gene in chromosome 18 (p-value<0.05) for the qualitative phenotype of schizophrenia was found (rs575682; ADNP2). The result was replicated for the quantitative phenotype of apparent onset, apparent onset of psychosis, and the first treatment age. We also found one significant SNP of ADNP2 gene in chromosome 18 (p-value<0.05) for the quantitative phenotype of any delusions. (rs575682; ADNP2) No SNPs were found for the quantitative phenotype of any hallucinations. Conclusion ：Our results showed that quantitative traits such as age of onset, any delusions, and any hallucinations could be continuous with qualitative trait in schizophrenia. However, a caution must be taken in interpreting these results because there were clear limitations in FBAT analyses which included nominal number of SNPs in the small incomplete pedigrees.