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Purpose@#We conducted a nationwide, multicenter, prospective registry study for newly diagnosed patients with peripheral T-cell lymphoma (PTCL) to better define the clinical characteristics, treatment patterns, survival outcomes, and the role of upfront autologous stem cell transplantation (ASCT) in these patients. @*Materials and Methods@#Patients with PTCL receiving chemotherapy with curative intent were registered and prospectively monitored. All patients were pathologically diagnosed with PTCL. @*Results@#A total of 191 patients with PTCL were enrolled in this prospective registry study. PTCL, not otherwise specified (PTCL-NOS) was the most common pathologic subtype (n=80, 41.9%), followed by angioimmunoblastic T-cell lymphoma (AITL) (n=60, 31.4%). With a median follow-up duration of 3.9 years, the 3-year progression-free survival (PFS) and overall survival (OS) rates were 39.5% and 60.4%, respectively. The role of upfront ASCT was evaluated in patients who were considered transplant-eligible (n=59). ASCT was performed as an upfront consolidative treatment in 32 (54.2%) of these patients. There were no significant differences in PFS and OS between the ASCT and non-ASCT groups for all patients (n=59) and for patients with PTCL-NOS (n=26). However, in patients with AITL, the ASCT group was associated with significantly better PFS than the non-ASCT group, although there was no significant difference in OS. @*Conclusion@#The current study demonstrated that the survival outcomes with the current treatment options remain poor for patients with PTCL-NOS. Upfront ASCT may provide a survival benefit for patients with AITL, but not PTCL-NOS.
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Purpose@#Prognostic Index for Natural Killer Lymphoma (PINK) is the most widely accepted prognostic model for patients withextranodal natural killer/T-cell lymphoma (ENKTL) treated with non-anthracycline–based therapy. We aimed to evaluate the prognostic implications of serum β-2 microglobulin (β2M) in the context of PINK and proposed a new prognostic model. @*Materials and Methods@#A total of 138 patients who were newly diagnosed with ENKTL and treated with non-anthracycline-based chemotherapy were identified. The cut-off value of high serum β2M was calculated by maximal-chi square methods (4.1 mg/L). A new prognostic model incorporating serum β2M into PINK was proposed and validated in an independent validation cohort (n=88). @*Results@#The patients’ median age was 53.5 years (range, 19 to 80 years). Patients with high serum β2M levels had significantly worse overall survival (OS) and progression-free survival (PFS). In multivariate analysis, high serum β2M was an independent adverse prognostic factor for OS. A new PINK-B (Prognostic Index for Natural Killer Lymphoma-serum β-2 microglobulin) model stratifiedpatients into three groups with distinct OS and PFS in the training cohort (3-year OS, 84.1% [95% confidence interval, 75.1 to 94.2], 46.8% [36.1 to 60.8] and 17.6% [6.3 to 49.2] for the low-, intermediate, and high-risk groups, respectively; 3-year PFS, 70.6% [59.4 to 83.8], 35.9% [25.9 to 49.8], and 7.35% [1.1 to 46.7] for the low-, intermediate-, and high-risk groups, respectively). The PINK-B model was further validated in an independent cohort. @*Conclusion@#Serum β2M is an independent prognostic factor for ENKTL patients. The new serum β2M-based prognostic model may be useful for identifying ultra-high-risk patients, and it can easily be adopted into daily clinical practice.
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Background/Aims@#The incidence of multiple myeloma (MM) in Korea is rapidly increasing. The diagnostic criteria of MM have been updated and novel therapeutic agents are available. This study explored the features of MM patients registered at Asan Medical Center (AMC) and the outcomes over the past 15 years. @*Methods@#Data were obtained from the AMC MM registry, which has been collecting the data of MM patients prospectively. The 774 MM patients included in our analysis were diagnosed from 2003, when thalidomide became available as a novel therapeutic agent, until April 2019. The 2-year survival rate of these patients was assessed. Patients were divided into two groups based on whether they were older or younger than 65 years, which is the cutoff age for the indication of autologous stem cell transplantation. Patients were also grouped according to the year of diagnosis: up to 2006, when bortezomib became available, and up to 2010, when the cost of lenalidomide was reimbursed. @*Results@#Patients < 65 years of age had better prognostic features, including a better performance, less advanced disease stage, and fewer abnormalities in their fluorescent in-situ hybridization (FISH) analysis results. A comparison of our Korean patients with patients registered in the Myeloma Related Disorder Registry data of Australia and New Zealand, showed ethnic discrepancies. The median overall survival of all patients was 3.7 years, with a 5-year survival rate of 41.8% and a 10-year survival rate of 23.4%. Survival progressively improved in patients diagnosed later. Age, performance status, renal function, C-reactive protein level, lactate dehydrogenase level, and cytogenetic findings were identified as significant prognostic factors. @*Conclusions@#This real-world survey revealed the clinical features and survival rates of patients at a tertiary Korean Hospital who were diagnosed with MM at the beginning of 21st century.
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The term “monoclonal gammopathy of clinical significance” (MGCS) refers to any plasma cell or B-cell clonal disorder that does not meet the current criteria for malignant disorders but produces a monoclonal protein that directly or indirectly results in organ damage. The most commonly affected organs are the kidneys, nerves, and skin. This review summarizes the current classification of MGCS and its diagnostic and treatment approaches.
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Background/Aims@#The first autologous peripheral blood stem cell transplantation (ASCT) in Korea was performed for a small-cell lung cancer patient at Asan Medical Center (AMC) in 1993. Recently, lymphoma and myeloma have been the main indications; there has been progress in the treatments for these lymphoid malignancies. We explored the real-world experience of ASCT for lymphoma and myeloma at AMC over a 25-year period. @*Methods@#We used the AMC ASCT registry, which has collected ASCT data prospectively since January 1993. Data for Hodgkin lymphoma, non-Hodgkin lymphoma, and multiple myeloma patients were analyzed. Patients transplanted up to December 2018 were included to assess adequate survival data. The ASCT time period was divided arbitrarily into 1994-1999, 2000-2009, and 2010-2018. In cases of multiple myeloma, we analyzed the 1st ASCT data only. @*Results@#Survival of these lymphoid malignancy patients after ASCT has progressively improved. The increase in survival may be related to advances in various medical skills supporting ASCT. However, overall survival has improved much more than progression-free survival. This suggests that better salvage therapies after ASCT failure have mainly affected the improvement in overall survival. The hematopoietic cell transplantation-specific comorbidity index could not be used as a survival indicator in this analysis. @*Conclusions@#This real-world experience study showed that the survival of lymphoid malignancy patients treated with ASCT has improved over the past 25 years.
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Purpose@#We aimed to investigate the prognostic value of serum β2-microglobulin for patients with Burkitt lymphoma (BL) and to propose a risk-stratifying classification system. @*Materials and Methods@#A prospective registry-based cohort study of BL patients treated with dose-intensive or effective dose-adjusted chemotherapies (n=81) was conducted. Survival outcomes were compared based on previously reported risk groups and/or serum β2-microglobulin levels. A risk-stratifying classification system incorporating serum β2-microglobulin levels was proposed and validated in an independent validation cohort (n=60). @*Results@#The median age was 47 years, and 57 patients (70.4%) were male. Patients with high serum β2-microglobulin levels (> 2 mg/L) had significantly worse progression-free survival (PFS) and overall survival (OS) (p 2 mg/L) was independently associated with a shorter PFS (hazards ratio [HR], 3.56; p=0.047) and OS (HR, 4.66; p=0.043). The new classification system incorporating the serum β2-microglobulin level allowed the stratification of patients into three distinct risk subgroups with 5-year OS rates of 100%, 89.5%, and 62.5%. In an independent cohort of BL, the system was validated by stratifying patients with different survival outcomes. @*Conclusion@#Serum β2-microglobulin level is an independent prognostic factor for BL patients. The proposed β2-microglobulin–based classification system could stratify patients with distinct survival outcomes, which may help define appropriate treatment approaches for individual patients.
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Background@#Follicular lymphoma (FL) is the second most common non-Hodgkin lymphoma (NHL) in Western countries. However, it is relatively rare in Asia. This study examined epidemiologic characteristics of FL in South Korea, with an emphasis on recent trends of increase in cases. @*Methods@#We retrospectively examined 239 cases of newly diagnosed FL at a large tertiary institution in Korea (Asan Medical Center, Seoul, Republic of Korea) between 2008 and 2017. Age-adjusted incidence rates and clinicopathological variables were analyzed, and joinpoint regression analysis was used to identify the changes. @*Results@#The age-adjusted incidence of FL significantly increased during the study period (p = .034), and the ratio of (relative incidence) patients with FL to patients with NHL increased from 4.28% to 9.35% in the same period. Over the 10-year study assessment duration, the proportion of patients with stage III/IV FL (p = .035) and expression of BCL2 (p = .022) or BCL6 (p = .039) significantly increased. From 2013–2017, the proportion of patients with highrisk Follicular Lymphoma International Prognostic Index (FLIPI) score increased (21.5% to 28.7%), whereas that of low-risk FLIPI decreased (55.4% to 38.6%), although those results were not statistically significant (p = .066). @*Conclusions@#We found an increasing incidence of FL, with a disproportionate increase in the incidence of high-stage disease and recent changes in the clinicopathologic features of the Korean patient population.
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Purpose@#We aimed to investigate the prognostic value of serum β2-microglobulin for patients with Burkitt lymphoma (BL) and to propose a risk-stratifying classification system. @*Materials and Methods@#A prospective registry-based cohort study of BL patients treated with dose-intensive or effective dose-adjusted chemotherapies (n=81) was conducted. Survival outcomes were compared based on previously reported risk groups and/or serum β2-microglobulin levels. A risk-stratifying classification system incorporating serum β2-microglobulin levels was proposed and validated in an independent validation cohort (n=60). @*Results@#The median age was 47 years, and 57 patients (70.4%) were male. Patients with high serum β2-microglobulin levels (> 2 mg/L) had significantly worse progression-free survival (PFS) and overall survival (OS) (p 2 mg/L) was independently associated with a shorter PFS (hazards ratio [HR], 3.56; p=0.047) and OS (HR, 4.66; p=0.043). The new classification system incorporating the serum β2-microglobulin level allowed the stratification of patients into three distinct risk subgroups with 5-year OS rates of 100%, 89.5%, and 62.5%. In an independent cohort of BL, the system was validated by stratifying patients with different survival outcomes. @*Conclusion@#Serum β2-microglobulin level is an independent prognostic factor for BL patients. The proposed β2-microglobulin–based classification system could stratify patients with distinct survival outcomes, which may help define appropriate treatment approaches for individual patients.
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Background@#Follicular lymphoma (FL) is the second most common non-Hodgkin lymphoma (NHL) in Western countries. However, it is relatively rare in Asia. This study examined epidemiologic characteristics of FL in South Korea, with an emphasis on recent trends of increase in cases. @*Methods@#We retrospectively examined 239 cases of newly diagnosed FL at a large tertiary institution in Korea (Asan Medical Center, Seoul, Republic of Korea) between 2008 and 2017. Age-adjusted incidence rates and clinicopathological variables were analyzed, and joinpoint regression analysis was used to identify the changes. @*Results@#The age-adjusted incidence of FL significantly increased during the study period (p = .034), and the ratio of (relative incidence) patients with FL to patients with NHL increased from 4.28% to 9.35% in the same period. Over the 10-year study assessment duration, the proportion of patients with stage III/IV FL (p = .035) and expression of BCL2 (p = .022) or BCL6 (p = .039) significantly increased. From 2013–2017, the proportion of patients with highrisk Follicular Lymphoma International Prognostic Index (FLIPI) score increased (21.5% to 28.7%), whereas that of low-risk FLIPI decreased (55.4% to 38.6%), although those results were not statistically significant (p = .066). @*Conclusions@#We found an increasing incidence of FL, with a disproportionate increase in the incidence of high-stage disease and recent changes in the clinicopathologic features of the Korean patient population.
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Clear-cell sarcoma (CCS) is a rare soft tissue sarcoma that usually develops in the lower extremities of young adults. CCS of the gastrointestinal tract is extremely rare. We report here the first case of CCS of the stomach in Korea. A 28-year-old female developed a large tumor of the stomach that was initially considered Ewing sarcoma, based on the results of a needle biopsy. She had paraneoplastic syndrome; fever, anemia, and hyperglycemia. Follow-up abdominal computed tomography after four-cycles of chemotherapy with vincristine-doxorubicin-cyclophosphamide or ifosfamide-etoposide showed no tumor shrinkage and revealed a fistula between the tumor and the gastric lumen. The infected tumor prompted debulking surgery, which resulted in improvement of her symptoms. The pathologic findings were consistent with CCS, and fluorescence in situ hybridization for EWS gene rearrangement was positive, confirming the diagnosis. This case highlights the importance of clinical suspicion for CCS in chemotherapy-refractory Ewing sarcoma.
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Adulto , Feminino , Humanos , Adulto Jovem , Anemia , Biópsia por Agulha , Diagnóstico , Tratamento Farmacológico , Febre , Fístula , Fluorescência , Seguimentos , Trato Gastrointestinal , Rearranjo Gênico , Hiperglicemia , Hibridização In Situ , Coreia (Geográfico) , Extremidade Inferior , Síndromes Paraneoplásicas , Sarcoma , Sarcoma de Células Claras , Sarcoma de Ewing , EstômagoRESUMO
BACKGROUND: The American Geriatric Society released the 2012 updated version of the Beers criteria with intentions to improve care of older adults by reducing their exposure to potentially inappropriate medications (PIMs). However, there have been no reports on the prevalence of PIMs prescriptions according to the 2012 version of Beers criteria in South Korea. METHODS: This is a retrospective study using medical records and code analysis of each PIM to survey the prevalence of PIMs prescriptions and common PIMs used for elderly patients. Locating the PIMs was carried out in all outpatients who visited Asan Medical Center from May 2012 to April 2013. Selection of PIMs was based on the 2012 updated version of the Beers criteria. RESULTS: A total of 652,192 outpatients older than 65 years visited our medical center during the study period and were analyzed. Among them, 33,810 (5.19%) received at least one PIM and 125,498 cases of PIM prescriptions were written. The percentage of the patients who received at least two kinds of PIMs concurrently was 33.14%. Common PIMs were tramadol (24.15%), clonazepam(11.51%), ibuprofen (10.02%), megesterol (9.80%), and amitriptyline (9.51%). CONCLUSION: Our study investigated the prevalence of PIMs prescription for Korean elderly outpatients in a single tertiary medical center. Compared to previous reports using the older version of the Beers criteria, our study showed a change in the priority of common PIMs.