Pub Date : 2024-04-01DOI: 10.3904/kjm.2024.99.2.116
Young Kyeong Seo, Taehee Kim, Yeong Hoon Kim, Yunmi Kim, H. Huh, Byeong Woo Kim
Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis predominantly involving medium- or small-sized arteries, typically of the kidneys and other internal organs. Given the rarity of PAN and the variable clinical presentation, diagnosis is challenging and, to date, no definitive diagnostic marker has been identified. A patient diagnosed with immunoglobulin A nephropathy was observed to exhibit deterioration in renal function. To determine whether new structural abnormalities had developed, computed tomography scans of the kidneys, ureters, and bladder were obtained. Both kidneys exhibited multiple cortical defects, and a renal angiogram was performed to determine the cause. Angiography revealed partial obliteration of the left distal renal artery branches and multifocal extensive infarctions in both kidneys, and the patient was diagnosed with renal-limited PAN. Following steroid monotherapy, an improvement in renal function was observed. We believe that this case report may be helpful to physicians who assess and treat patients with suspected renal-limited PAN.
结节性多动脉炎(PAN)是一种全身性坏死性血管炎,主要累及中型或小型动脉,通常发生在肾脏和其他内脏器官。鉴于 PAN 的罕见性和多变的临床表现,诊断具有挑战性,迄今为止尚未发现明确的诊断标志物。一名被诊断为免疫球蛋白 A 肾病的患者被观察到肾功能恶化。为了确定是否出现了新的结构异常,患者接受了肾脏、输尿管和膀胱的计算机断层扫描。两个肾脏都出现了多处皮质缺损,为确定病因,进行了肾血管造影检查。血管造影显示左肾动脉远端分支部分闭塞,双肾多灶性广泛梗死,患者被诊断为肾局限性 PAN。在接受类固醇单药治疗后,患者的肾功能有所改善。我们相信,本病例报告可能对评估和治疗疑似肾局限性 PAN 患者的医生有所帮助。
{"title":"Polyarteritis Nodosa Confined to the Kidneys in a Patient with Proteinuria and Mild Renal Impairment","authors":"Young Kyeong Seo, Taehee Kim, Yeong Hoon Kim, Yunmi Kim, H. Huh, Byeong Woo Kim","doi":"10.3904/kjm.2024.99.2.116","DOIUrl":"https://doi.org/10.3904/kjm.2024.99.2.116","url":null,"abstract":"Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis predominantly involving medium- or small-sized arteries, typically of the kidneys and other internal organs. Given the rarity of PAN and the variable clinical presentation, diagnosis is challenging and, to date, no definitive diagnostic marker has been identified. A patient diagnosed with immunoglobulin A nephropathy was observed to exhibit deterioration in renal function. To determine whether new structural abnormalities had developed, computed tomography scans of the kidneys, ureters, and bladder were obtained. Both kidneys exhibited multiple cortical defects, and a renal angiogram was performed to determine the cause. Angiography revealed partial obliteration of the left distal renal artery branches and multifocal extensive infarctions in both kidneys, and the patient was diagnosed with renal-limited PAN. Following steroid monotherapy, an improvement in renal function was observed. We believe that this case report may be helpful to physicians who assess and treat patients with suspected renal-limited PAN.","PeriodicalId":517046,"journal":{"name":"The Korean Journal of Medicine","volume":"261 ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140759097","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-04-01DOI: 10.3904/kjm.2024.99.2.84
Sang-Heum Park, Samel Park, Jin Young Kim, Hyeon Ah Lee, Sang Mi Lee, Tae Hoon Lee, Sang Byung Bae, Sung Hae Chang, Si Hyong Jang, Sung Wan Chun, Jong Ho Moon
In Harrison’s Principles of Internal Medicine, human understanding is emphasized as one of three necessary characteristics that a physician must have. Inflammation, which is caused by inflammatory inducers (inf-ids), is a fundamental feature of disease at the cellular and molecular levels. Inflammation protects the body, but excessive or prolonged inflammation can be damaging and can cause disease. Humans are repeatedly exposed to external and internal environmental factors that generate inf-ids throughout their lives. External environmental factors include microbial and non-microbial inf-ids, as well as stressors that inevitably arise during social interactions. Internal environmental factors include the adaptive physiological response that is present from birth. Inf-ids may also be produced by the four-step habit loop, which consists of a cue (e.g., stressor), emotions, routine act (adaptive response), and a reward. Immune cells in the circulatory system and in tissues may have positive and negative effects in inflammatory responses. However, low-grade inflammation may be difficult to detect. We propose a model of disease development that integrates external and internal environmental factors from the perspective of human understanding.
{"title":"Human Understanding is Expected of the Physician: Proposing a Model of Disease Development","authors":"Sang-Heum Park, Samel Park, Jin Young Kim, Hyeon Ah Lee, Sang Mi Lee, Tae Hoon Lee, Sang Byung Bae, Sung Hae Chang, Si Hyong Jang, Sung Wan Chun, Jong Ho Moon","doi":"10.3904/kjm.2024.99.2.84","DOIUrl":"https://doi.org/10.3904/kjm.2024.99.2.84","url":null,"abstract":"In Harrison’s Principles of Internal Medicine, human understanding is emphasized as one of three necessary characteristics that a physician must have. Inflammation, which is caused by inflammatory inducers (inf-ids), is a fundamental feature of disease at the cellular and molecular levels. Inflammation protects the body, but excessive or prolonged inflammation can be damaging and can cause disease. Humans are repeatedly exposed to external and internal environmental factors that generate inf-ids throughout their lives. External environmental factors include microbial and non-microbial inf-ids, as well as stressors that inevitably arise during social interactions. Internal environmental factors include the adaptive physiological response that is present from birth. Inf-ids may also be produced by the four-step habit loop, which consists of a cue (e.g., stressor), emotions, routine act (adaptive response), and a reward. Immune cells in the circulatory system and in tissues may have positive and negative effects in inflammatory responses. However, low-grade inflammation may be difficult to detect. We propose a model of disease development that integrates external and internal environmental factors from the perspective of human understanding.","PeriodicalId":517046,"journal":{"name":"The Korean Journal of Medicine","volume":"100 ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140768601","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-04-01DOI: 10.3904/kjm.2024.99.2.96
Yun-Gyoo Lee, Hyun-Il Gil, Soo Jeong Kim, Hyunjoo Lee, Heerim Nam, Soo-Youn Ham, Du-Young Kang
Lung cancer is the leading cause of cancer death in Republic of Korea. After their initial diagnosis, only 10-20% of patients with advanced non-small cell lung cancer (NSCLC) survive for 5 years of longer. Given enormous advances in therapeutics such as novel targeted therapies and immunotherapies, survival rates are improving for advanced patients with NSCLC; 5-year survival rates range from 15% to 50%, contingent upon the biomarker. Detection of the specific molecular alteration as biomarker is thus crucial for identifying subgroups of NSCLC that contain therpapeutically targetable oncogenic drivers. This review examines the process of diagnosing lung adenocarcinoma with dominant biomarkers in order to customize treatment with appropriate targeted therapy.
{"title":"Targeted Therapy of Advanced Non-Small Cell Lung Cancer","authors":"Yun-Gyoo Lee, Hyun-Il Gil, Soo Jeong Kim, Hyunjoo Lee, Heerim Nam, Soo-Youn Ham, Du-Young Kang","doi":"10.3904/kjm.2024.99.2.96","DOIUrl":"https://doi.org/10.3904/kjm.2024.99.2.96","url":null,"abstract":"Lung cancer is the leading cause of cancer death in Republic of Korea. After their initial diagnosis, only 10-20% of patients with advanced non-small cell lung cancer (NSCLC) survive for 5 years of longer. Given enormous advances in therapeutics such as novel targeted therapies and immunotherapies, survival rates are improving for advanced patients with NSCLC; 5-year survival rates range from 15% to 50%, contingent upon the biomarker. Detection of the specific molecular alteration as biomarker is thus crucial for identifying subgroups of NSCLC that contain therpapeutically targetable oncogenic drivers. This review examines the process of diagnosing lung adenocarcinoma with dominant biomarkers in order to customize treatment with appropriate targeted therapy.","PeriodicalId":517046,"journal":{"name":"The Korean Journal of Medicine","volume":"303 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140781216","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-04-01DOI: 10.3904/kjm.2024.99.2.69
Jong Sun Park
Hypersensitivity pneumonitis (HP) is an inflammatory and fibrosing interstitial lung disease caused by inhaled antigens in susceptible individuals. Various environmental materials can serve as inciting agents of HP. Recent guidelines have defined typical radiologic and histopathologic findings of HP, classifying it into nonfibrotic and fibrotic HP categories, as this classification better correlates with clinical outcomes. Diagnosing HP poses challenges, necessitating multidisciplinary discussions based on clinical assessments and radiologic or histopathological features. The cornerstone of treatment lies in avoiding the causative antigen. Oral glucocorticoids serve as the initial treatment for symptomatic HP. Immunosuppressants may be employed in patients who do not respond to initial treatment, and antifibrotics could be considered for patients displaying progressive fibrosis. This review outlines the clinical features, diagnostic evaluation, and treatment of HP.
超敏性肺炎(HP)是一种由易感人群吸入抗原引起的炎症性纤维化间质性肺病。各种环境物质都可能成为过敏性肺炎的诱发因素。最近的指南定义了 HP 的典型放射学和组织病理学结果,将其分为非纤维化和纤维化 HP 两类,因为这种分类与临床结果更相关。HP 的诊断具有挑战性,需要根据临床评估和放射学或组织病理学特征进行多学科讨论。治疗的基石在于避免使用致病抗原。口服糖皮质激素是治疗无症状 HP 的初始疗法。对初始治疗无效的患者可使用免疫抑制剂,显示进行性纤维化的患者可考虑使用抗纤维化药物。本综述概述了 HP 的临床特征、诊断评估和治疗方法。
{"title":"Diagnosis, Clinical Course and Treatment of Hypersensitivity Pneumonitis","authors":"Jong Sun Park","doi":"10.3904/kjm.2024.99.2.69","DOIUrl":"https://doi.org/10.3904/kjm.2024.99.2.69","url":null,"abstract":"Hypersensitivity pneumonitis (HP) is an inflammatory and fibrosing interstitial lung disease caused by inhaled antigens in susceptible individuals. Various environmental materials can serve as inciting agents of HP. Recent guidelines have defined typical radiologic and histopathologic findings of HP, classifying it into nonfibrotic and fibrotic HP categories, as this classification better correlates with clinical outcomes. Diagnosing HP poses challenges, necessitating multidisciplinary discussions based on clinical assessments and radiologic or histopathological features. The cornerstone of treatment lies in avoiding the causative antigen. Oral glucocorticoids serve as the initial treatment for symptomatic HP. Immunosuppressants may be employed in patients who do not respond to initial treatment, and antifibrotics could be considered for patients displaying progressive fibrosis. This review outlines the clinical features, diagnostic evaluation, and treatment of HP.","PeriodicalId":517046,"journal":{"name":"The Korean Journal of Medicine","volume":"136 ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140772094","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-04-01DOI: 10.3904/kjm.2024.99.2.104
H. Jeon, Hyuk Soon Choi
Helicobacter pylori (H. pylori) is a bacterium that colonizes the human stomach, leading to various gastrointestinal diseases including gastritis, peptic ulcers, and gastric cancer. There is no gold standard test that relies entirely on one method in H. pylori diagnosis. We must be aware of the pros and cons of various testing methods to perform an appropriate test according to the situation. Accurate diagnosis and eradication therapy are essential for disease management. Diagnostic methods include invasive techniques like tissue biopsy and rapid urease test, as well as non-invasive tests such as urea breath test, serology test, and stool antigen test. Each method has its advantages and limitations, requiring careful consideration in clinical practice. Understanding these diagnostic tools is crucial for effective H. pylori management and prevention of associated complications.
{"title":"Various Diagnostic Methods for Helicobacter pylori Infection","authors":"H. Jeon, Hyuk Soon Choi","doi":"10.3904/kjm.2024.99.2.104","DOIUrl":"https://doi.org/10.3904/kjm.2024.99.2.104","url":null,"abstract":"Helicobacter pylori (H. pylori) is a bacterium that colonizes the human stomach, leading to various gastrointestinal diseases including gastritis, peptic ulcers, and gastric cancer. There is no gold standard test that relies entirely on one method in H. pylori diagnosis. We must be aware of the pros and cons of various testing methods to perform an appropriate test according to the situation. Accurate diagnosis and eradication therapy are essential for disease management. Diagnostic methods include invasive techniques like tissue biopsy and rapid urease test, as well as non-invasive tests such as urea breath test, serology test, and stool antigen test. Each method has its advantages and limitations, requiring careful consideration in clinical practice. Understanding these diagnostic tools is crucial for effective H. pylori management and prevention of associated complications.","PeriodicalId":517046,"journal":{"name":"The Korean Journal of Medicine","volume":"54 ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140770790","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-02-01DOI: 10.3904/kjm.2024.99.1.50
Jung-Ah Kim, Hyunjoo Dong, E. Lee, Jongtak Jung, Yae Jee Baek, Tae Hyong Kim, Tae Youn Choi
Nocardiosis is uncommon. Immunocompromising conditions predispose individuals to pulmonary and disseminated nocardiosis of the brain, skin, and subcutaneous tissues. The most common pathogens are Nocardia cyriacigeorgica, Nocardia nova, and Nocardia farcinica. The speciation of Nocardia to determine antimicrobial susceptibility is difficult using traditional biochemical methods. Here, we report the case of a 73-year-old man with chronic obstructive lung disease who developed a rapidly progressing intramuscular abscess around the left hip and thigh. Within 3 days, the lesions progressed to an epidural abscess at the L4 to S1 level. Although he was treated with broad-spectrum antibiotics and extensive incision and drainage, he died of rapidly progressive respiratory failure. Nocardia abscessus (N. abscessus) was identified in pus samples using matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS). This case shows that the diagnosis of an intramuscular abscess caused by N. abscessus is challenging and that using MALDI-TOF MS may facilitate the diagnosis and ensure appropriate treatment.
诺卡菌病并不常见。免疫力低下的人容易患肺部和脑部、皮肤和皮下组织的播散性诺卡氏杆菌病。最常见的致病菌是 cyriacigeorgica 诺卡氏菌、Nocardia nova 诺卡氏菌和 Nocardia farcinica 诺卡氏菌。使用传统的生化方法很难确定诺卡氏菌的种类以确定其抗菌药敏感性。在此,我们报告了一例患有慢性阻塞性肺病的 73 岁男性病例,他的左臀部和大腿周围出现了进展迅速的肌肉内脓肿。3 天内,病变发展为 L4 至 S1 水平的硬膜外脓肿。虽然他接受了广谱抗生素治疗和大面积切开引流,但还是死于快速进展的呼吸衰竭。使用基质辅助激光解吸/电离飞行时间质谱法(MALDI-TOF MS)在脓液样本中鉴定出了脓肿诺卡氏菌(N. abscessus)。该病例表明,诊断由诺卡氏脓肿引起的肌肉内脓肿具有挑战性,而使用 MALDI-TOF MS 可以促进诊断并确保适当的治疗。
{"title":"Multiple Intramuscular Abscesses Caused by Nocardia abscessus in a Patient with Chronic Obstructive Lung Disease: Clinical Microbiology Considerations","authors":"Jung-Ah Kim, Hyunjoo Dong, E. Lee, Jongtak Jung, Yae Jee Baek, Tae Hyong Kim, Tae Youn Choi","doi":"10.3904/kjm.2024.99.1.50","DOIUrl":"https://doi.org/10.3904/kjm.2024.99.1.50","url":null,"abstract":"Nocardiosis is uncommon. Immunocompromising conditions predispose individuals to pulmonary and disseminated nocardiosis of the brain, skin, and subcutaneous tissues. The most common pathogens are Nocardia cyriacigeorgica, Nocardia nova, and Nocardia farcinica. The speciation of Nocardia to determine antimicrobial susceptibility is difficult using traditional biochemical methods. Here, we report the case of a 73-year-old man with chronic obstructive lung disease who developed a rapidly progressing intramuscular abscess around the left hip and thigh. Within 3 days, the lesions progressed to an epidural abscess at the L4 to S1 level. Although he was treated with broad-spectrum antibiotics and extensive incision and drainage, he died of rapidly progressive respiratory failure. Nocardia abscessus (N. abscessus) was identified in pus samples using matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS). This case shows that the diagnosis of an intramuscular abscess caused by N. abscessus is challenging and that using MALDI-TOF MS may facilitate the diagnosis and ensure appropriate treatment.","PeriodicalId":517046,"journal":{"name":"The Korean Journal of Medicine","volume":"1 4","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139893999","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-02-01DOI: 10.3904/kjm.2024.99.1.50
Jung-Ah Kim, Hyunjoo Dong, E. Lee, Jongtak Jung, Yae Jee Baek, Tae Hyong Kim, Tae Youn Choi
Nocardiosis is uncommon. Immunocompromising conditions predispose individuals to pulmonary and disseminated nocardiosis of the brain, skin, and subcutaneous tissues. The most common pathogens are Nocardia cyriacigeorgica, Nocardia nova, and Nocardia farcinica. The speciation of Nocardia to determine antimicrobial susceptibility is difficult using traditional biochemical methods. Here, we report the case of a 73-year-old man with chronic obstructive lung disease who developed a rapidly progressing intramuscular abscess around the left hip and thigh. Within 3 days, the lesions progressed to an epidural abscess at the L4 to S1 level. Although he was treated with broad-spectrum antibiotics and extensive incision and drainage, he died of rapidly progressive respiratory failure. Nocardia abscessus (N. abscessus) was identified in pus samples using matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS). This case shows that the diagnosis of an intramuscular abscess caused by N. abscessus is challenging and that using MALDI-TOF MS may facilitate the diagnosis and ensure appropriate treatment.
诺卡菌病并不常见。免疫力低下的人容易患肺部和脑部、皮肤和皮下组织的播散性诺卡氏杆菌病。最常见的致病菌是 cyriacigeorgica 诺卡氏菌、Nocardia nova 诺卡氏菌和 Nocardia farcinica 诺卡氏菌。使用传统的生化方法很难确定诺卡氏菌的种类以确定其抗菌药敏感性。在此,我们报告了一例患有慢性阻塞性肺病的 73 岁男性病例,他的左臀部和大腿周围出现了进展迅速的肌肉内脓肿。3 天内,病变发展为 L4 至 S1 水平的硬膜外脓肿。虽然他接受了广谱抗生素治疗和大面积切开引流,但还是死于快速进展的呼吸衰竭。使用基质辅助激光解吸/电离飞行时间质谱法(MALDI-TOF MS)在脓液样本中鉴定出了脓肿诺卡氏菌(N. abscessus)。该病例表明,诊断由诺卡氏脓肿引起的肌肉内脓肿具有挑战性,而使用 MALDI-TOF MS 可以促进诊断并确保适当的治疗。
{"title":"Multiple Intramuscular Abscesses Caused by Nocardia abscessus in a Patient with Chronic Obstructive Lung Disease: Clinical Microbiology Considerations","authors":"Jung-Ah Kim, Hyunjoo Dong, E. Lee, Jongtak Jung, Yae Jee Baek, Tae Hyong Kim, Tae Youn Choi","doi":"10.3904/kjm.2024.99.1.50","DOIUrl":"https://doi.org/10.3904/kjm.2024.99.1.50","url":null,"abstract":"Nocardiosis is uncommon. Immunocompromising conditions predispose individuals to pulmonary and disseminated nocardiosis of the brain, skin, and subcutaneous tissues. The most common pathogens are Nocardia cyriacigeorgica, Nocardia nova, and Nocardia farcinica. The speciation of Nocardia to determine antimicrobial susceptibility is difficult using traditional biochemical methods. Here, we report the case of a 73-year-old man with chronic obstructive lung disease who developed a rapidly progressing intramuscular abscess around the left hip and thigh. Within 3 days, the lesions progressed to an epidural abscess at the L4 to S1 level. Although he was treated with broad-spectrum antibiotics and extensive incision and drainage, he died of rapidly progressive respiratory failure. Nocardia abscessus (N. abscessus) was identified in pus samples using matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS). This case shows that the diagnosis of an intramuscular abscess caused by N. abscessus is challenging and that using MALDI-TOF MS may facilitate the diagnosis and ensure appropriate treatment.","PeriodicalId":517046,"journal":{"name":"The Korean Journal of Medicine","volume":"16 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139897635","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-02-01DOI: 10.3904/kjm.2024.99.1.37
Hyungwoo Cho, Jung Yong Hong, Daeho Lee, S. Kim, Kyoungmin Lee, E. Kang, Sunjong Lee, J. Park, Jeong Hoon Kim, Jin-Sook Ryu, J. Huh, C. Suh
Background/Aims: In Korea, the incidence of primary diffuse large B-cell lymphoma of the central nervous system (PCNSL) is increasing and autologous stem cell transplantation (ASCT) has improved the survival of younger patients. We explored our real-world experience with PCNSL at Asan Medical Center (AMC).Methods: We used the AMC lymphoma registry to collect patient data prospectively. We analyzed 279 patients diagnosed from 2002 until August 2019.Results: The PCNSL incidence at AMC increased progressively and comprised 7.4-8.9% of new non-Hodgkin lymphoma patients annually during the most recent 4 years. The median age was 60 years (range, 17-85) and males comprised 55%. Patients under 65 years of age (n = 183) had no significant differences in characteristics compared to those aged 65 years or over, with the exception of less occipital lobe involvement and lower beta-2 microglobulin levels. Rituximab, methotrexate, procarbazine, and vincristine (R-MPV) combination induction had the best overall response, of 95%. The median overall survival was 3.8 years with 5- and 10-year survival rates of 41.5% and 30.2%, respectively. Survival was better in younger patients and those treated with ASCT. Thiotepa, busulfan, and cytoxan (TBC) conditioning chemotherapy had better survival than other combinations. The International Extranodal Lymphoma Study Group and Memorial Sloan Kettering Cancer Center prognostic score systems were valid in this cohort. Age and performance status were independent prognostic factors. Exclusive extra-central nervous system failure occurred in six patients (5.6%) among 107 failures.Conclusions: The incidence of PCNSL is rising. R-MPV induction therapy followed by ASCT with TBC has improved the survival of young, fit PCNSL patients.
{"title":"Single-Center Real-World Experience with Primary Central Nervous System Lymphoma in the 21st Century","authors":"Hyungwoo Cho, Jung Yong Hong, Daeho Lee, S. Kim, Kyoungmin Lee, E. Kang, Sunjong Lee, J. Park, Jeong Hoon Kim, Jin-Sook Ryu, J. Huh, C. Suh","doi":"10.3904/kjm.2024.99.1.37","DOIUrl":"https://doi.org/10.3904/kjm.2024.99.1.37","url":null,"abstract":"Background/Aims: In Korea, the incidence of primary diffuse large B-cell lymphoma of the central nervous system (PCNSL) is increasing and autologous stem cell transplantation (ASCT) has improved the survival of younger patients. We explored our real-world experience with PCNSL at Asan Medical Center (AMC).Methods: We used the AMC lymphoma registry to collect patient data prospectively. We analyzed 279 patients diagnosed from 2002 until August 2019.Results: The PCNSL incidence at AMC increased progressively and comprised 7.4-8.9% of new non-Hodgkin lymphoma patients annually during the most recent 4 years. The median age was 60 years (range, 17-85) and males comprised 55%. Patients under 65 years of age (n = 183) had no significant differences in characteristics compared to those aged 65 years or over, with the exception of less occipital lobe involvement and lower beta-2 microglobulin levels. Rituximab, methotrexate, procarbazine, and vincristine (R-MPV) combination induction had the best overall response, of 95%. The median overall survival was 3.8 years with 5- and 10-year survival rates of 41.5% and 30.2%, respectively. Survival was better in younger patients and those treated with ASCT. Thiotepa, busulfan, and cytoxan (TBC) conditioning chemotherapy had better survival than other combinations. The International Extranodal Lymphoma Study Group and Memorial Sloan Kettering Cancer Center prognostic score systems were valid in this cohort. Age and performance status were independent prognostic factors. Exclusive extra-central nervous system failure occurred in six patients (5.6%) among 107 failures.Conclusions: The incidence of PCNSL is rising. R-MPV induction therapy followed by ASCT with TBC has improved the survival of young, fit PCNSL patients.","PeriodicalId":517046,"journal":{"name":"The Korean Journal of Medicine","volume":"3 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139897546","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-02-01DOI: 10.3904/kjm.2024.99.1.11
Junshik Hong
Myelodysplastic syndrome (MDS) is a heterogeneous disorder with diverse prognoses influenced by cytopenias, genetic variants, and myeloblast proportions in the bone marrow. Accurate prognosis prediction and tailored treatment plans are essential. The International Prognostic Scoring System-Molecular (IPSS-M), which additionally reflects the impact of MDS-related genetic mutations to the clinical and laboratory information, is anticipated to offer superior prognostic accuracy compared to existing systems like the Revised International Prognostic Scoring System (IPSS-R). Despite its statistical complexity, its web-based calculation and ease of discussing results with patients using intuitive data sets provide notable advantages. Progress in MDS treatment, exemplified by effective anemia correction with an erythropoiesis-maturation agent in SF3B1-mutated cases and efforts to refine poor prognoses in TP53-mutated cases, reflects the evolving landscape of genetic-based interventions in MDS. Advancements in genetic diagnostic technology, combined with enhanced knowledge of the bone marrow niche, are anticipated to lead to significant improvement in MDS treatment outcomes in the future.
{"title":"Diagnosis and Treatment of Myelodysplastic Syndrome in the Era of Genetic Testing","authors":"Junshik Hong","doi":"10.3904/kjm.2024.99.1.11","DOIUrl":"https://doi.org/10.3904/kjm.2024.99.1.11","url":null,"abstract":"Myelodysplastic syndrome (MDS) is a heterogeneous disorder with diverse prognoses influenced by cytopenias, genetic variants, and myeloblast proportions in the bone marrow. Accurate prognosis prediction and tailored treatment plans are essential. The International Prognostic Scoring System-Molecular (IPSS-M), which additionally reflects the impact of MDS-related genetic mutations to the clinical and laboratory information, is anticipated to offer superior prognostic accuracy compared to existing systems like the Revised International Prognostic Scoring System (IPSS-R). Despite its statistical complexity, its web-based calculation and ease of discussing results with patients using intuitive data sets provide notable advantages. Progress in MDS treatment, exemplified by effective anemia correction with an erythropoiesis-maturation agent in SF3B1-mutated cases and efforts to refine poor prognoses in TP53-mutated cases, reflects the evolving landscape of genetic-based interventions in MDS. Advancements in genetic diagnostic technology, combined with enhanced knowledge of the bone marrow niche, are anticipated to lead to significant improvement in MDS treatment outcomes in the future.","PeriodicalId":517046,"journal":{"name":"The Korean Journal of Medicine","volume":"5 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139893760","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Malignant hilar biliary obstruction (MHO), an aggressive type of perihilar biliary obstruction caused by cholangiocarcinoma, gallbladder cancer, or other metastatic malignancies, has a poor prognosis. Surgical resection is the only curative treatment for biliary malignancies. However, most patients with MHO cannot undergo surgery upon presentation because of their advanced inoperable state or poor performance resulting from old age or comorbid diseases. Therefore, palliative biliary drainage is required to improve symptomatic jaundice and quality of life. Preoperative biliary drainage is controversial in resectable cases of MHO. Preoperative biliary drainage should be considered according to specific selection criteria. Palliative drainage is currently the mainstay of symptomatic treatment. Compared with percutaneous access, primary endoscopic palliation using plastic or metal stents has recently shown higher technical feasibility and clinical success without increasing the frequency of adverse events, even in high-degree MHO. However, the use of stents still has numerous limitations, including challenges in determining the optimal type of stent, number of stents, deployment method, and additional local therapies. Therefore, this report presents the current optimal endoscopic drainage status for MHO based on recent guidelines and published literature.
{"title":"Trends of Endoscopic Palliation for Advanced Malignant Hilar Biliary Obstruction","authors":"Tae Hoon Lee","doi":"10.3904/kjm.2024.99.1.4","DOIUrl":"https://doi.org/10.3904/kjm.2024.99.1.4","url":null,"abstract":"Malignant hilar biliary obstruction (MHO), an aggressive type of perihilar biliary obstruction caused by cholangiocarcinoma, gallbladder cancer, or other metastatic malignancies, has a poor prognosis. Surgical resection is the only curative treatment for biliary malignancies. However, most patients with MHO cannot undergo surgery upon presentation because of their advanced inoperable state or poor performance resulting from old age or comorbid diseases. Therefore, palliative biliary drainage is required to improve symptomatic jaundice and quality of life. Preoperative biliary drainage is controversial in resectable cases of MHO. Preoperative biliary drainage should be considered according to specific selection criteria. Palliative drainage is currently the mainstay of symptomatic treatment. Compared with percutaneous access, primary endoscopic palliation using plastic or metal stents has recently shown higher technical feasibility and clinical success without increasing the frequency of adverse events, even in high-degree MHO. However, the use of stents still has numerous limitations, including challenges in determining the optimal type of stent, number of stents, deployment method, and additional local therapies. Therefore, this report presents the current optimal endoscopic drainage status for MHO based on recent guidelines and published literature.","PeriodicalId":517046,"journal":{"name":"The Korean Journal of Medicine","volume":"27 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139897417","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}