Background: Hodgkin's lymphoma (HL) constitutes 10%–20% of all malignant lymphomas and has a high cure rate (5-year survival, around 90%). Recently, interest has increased concerning preventing secondary complications (secondary cancer, endocrine disorders) in long-term survivors. We aimed to study the epidemiologic features and therapeutic outcomes of HL in children, adolescents, and young adults in Korea. Methods: We performed a multicenter, retrospective study of 224 patients aged < 25 years diagnosed with HL at 22 participating institutes in Korea from January 2007 to August 2016. Results: A higher percentage of males was diagnosed at a younger age. Nodular sclerosis histopathological HL subtype was most common, followed by mixed cellularity subtype.Eighty-one (36.2%), 101 (45.1%), and 42 (18.8%) patients were classified into low, intermediate, and high-risk groups, respectively. Doxorubicin, bleomycin, vinblastine, dacarbazine was the most common protocol (n = 102, 45.5%). Event-free survival rate was 86.0% ± 2.4%, while five-year overall survival (OS) rate was 96.1% ± 1.4%: 98.7% ± 1.3%, 97.7% ± 1.6%, and 86.5% ± 5.6% in the low, intermediate, and high-risk groups, respectively (P = 0.021). Five-year OS was worse in patients with B-symptoms, stage IV disease, highrisk, splenic involvement, extra-nodal lymphoma, and elevated lactate dehydrogenase level.In multivariate analysis, B-symptoms and extra-nodal involvement were prognostic factors for poor OS. Late complications of endocrine disorders and secondary malignancy were observed in 17 and 6 patients, respectively. Conclusion This is the first study on the epidemiology and treatment outcomes of HL in children, adolescents, and young adults in Korea. Future prospective studies are indicated to develop therapies that minimize treatment toxicity while maximizing cure rates in children, adolescents, and young adults with HL.

BACKGROUND: Clinical trials that utilize imaging findings as surrogate endpoints are considered to be cost-effective. However, unlike numeric data, magnetic resonance imaging (MRI) findings are not quantifiable. Thus, we have begun to develop a software package that is able to convert qualitative MRI findings into quantifiable data. METHODS: Computer software (DUIH_Image) was created with which every patient's MRI data can be registered on a standard brain template. Interuser and intrauser reliabilities for the registration were measured, and then a proof-of-principle experiment was conducted to determine whether the system could identify factors that were associated with a greater National Institutes of Health Stroke Scale (NIHSS) score at admission. We studied 40 consecutive patients [65.1+/-14.2 years old (mean+/-SD); 22 males and 18 females] with first-ever acute lacunar infarction of the corona radiata, who were divided into two groups according to their NIHSS score (i.e., low: 0-2; high: > or =3). The following parameters were compared between these two groups: (1) data retrieved from clinical profiles, including demographic and risk factor variables; and (2) accumulated diffusion MRI lesions mapped on a standard template. RESULTS: Modest levels of interuser and intrauser reliability were observed (p<0.05, R(2)=0.63-0.84, Pearson correlations). Regarding the clinical profiles, no significant difference was found for the numeric data sets or infarct size between the two groups. However, on the accumulated lesion map image, the lesion area that overlapped the most was located more posterolaterally in the high NIHSS score group than in the low NIHSS score group. CONCLUSIONS: In this pilot study we have demonstrated the potential usefulness of the DUIH_Image software. We plan to update this software to enable its utilization in actual clinical trials.
Biomarkers ; Brain ; Computer Systems ; Diffusion Magnetic Resonance Imaging ; Humans ; Magnetic Resonance Imaging ; Male ; National Institutes of Health (U.S.) ; Pilot Projects ; Risk Factors ; Software ; Stroke ; Stroke, Lacunar

BACKGROUND: The aim of this study was to investigate the prevalence, clinical and laboratory findings of hereditary hemolytic anemia (HHA) in Korea from 1997 to 2006 and to develop the appropriate diagnostic approach for HHA. METHODS: By the use of questionnaires, information on the clinical and laboratory findings ofHHA diagnosed from 1997 to 2006 in Korea was collected and analyzed retrospectively. A total of 431 cases were enrolled in this study from 46 departments of 35 hospitals. RESULTS: The overall frequency of HHA did not change through the 10-year period for pediatrics but did show an increasing tendency for internal medicine. The overall male to female sex ratio did not show sex predominance (1.17:1), but a significant male predominance with a ratio of 1.49:1 was seen for pediatrics while a significant female predominance with a ratio of 1:1.97 was seen forinternal medicine. Of the total cases, 74.2% (282/431) were diagnosed before the age of 15 years. The etiologies of HHA were classified as red cell membrane defects, hemoglobinopathies, red cell enzyme deficiencies and unknown causes. There were 382 cases (88.6%) of red cell membrane defects with 376 cases (87.2%) of hereditary spherocytosis and 6 cases (1.4%) of hereditary elliptocytosis, 20 cases (4.6%) of hemoglobinopathies with 18 cases (4.2%) of beta-thalassemia, a case (0.2%) of alpha-thalassemia and a case (0.2%) of Hemoglobin Madrid, 7 cases (1.6%) of red cell enzyme deficiencies with 5 cases (1.2%) of glucose-6- phosphate dehydrogenase (G-6-PD) deficiency, a case (0.2%) of pyruvate kinase (PK) deficiency and a case (0.2%) of enolase deficiency, and 22 cases (5.1%) of unknown causes. The most common chief complaint in pediatric patients was pallor and that in adult patients was jaundice. In the red cell membrane defect group of patients, the level of hemoglobin was significantly higher than in adult patients. The mean corpuscular volume, mean corpuscular hemoglobin, corrected reticulocyte count, total and indirect bilirubin level and lactate dehydrogenase levels in the hemoglobinopathy group of patients were significantly lower than the values in the red cell membrane defect group of patients. The mean concentration of G-6-PD was 0.8+/-0.7U/1012RBC in the G-6-PD deficient patients, PK was 1.7U/1010 RBC in the PK deficient patient, and the level of enolase was 0.04U/g of Hb in the enolase deficient patient. CONCLUSION: The most prevalent cause of HHA in Korea during 1997 to 2006 was hereditary spherocytosis, but HHA by other causes such as hemoglobinopathy and red cell enzyme deficiency gradually increased with the development of molecular diagnostic methods and increasing general interest. However, the etiologies of HHA need to be pursued further in 5.1% of the patients. An systematic standard diagnostic approach is needed in a nationwide prospective study for correct diagnoses and appropriate management of HHA.
Adult ; alpha-Thalassemia ; Anemia, Hemolytic, Congenital* ; beta-Thalassemia ; Bilirubin ; Cell Membrane ; Diagnosis ; Elliptocytosis, Hereditary ; Erythrocyte Indices ; Female ; Hemoglobinopathies ; Humans ; Internal Medicine ; Jaundice ; Korea* ; L-Lactate Dehydrogenase ; Male ; Oxidoreductases ; Pallor ; Pathology, Molecular ; Pediatrics ; Phosphopyruvate Hydratase ; Prevalence ; Pyruvate Kinase ; Reticulocyte Count ; Retrospective Studies* ; Sex Ratio ; Surveys and Questionnaires

BACKGROUND: Positive rate of HCV RNA in anti-HCV immunoblot indeterminate samples from Korean blood donors was investigated in this study. Futhermore, reactivity of enzyme immunoassay kits was evaluated according to patterns of band reactivity and RNA positivity of indeterminate samples. METHODS: HCV RNA was tested from a total of 180 samples that had been repeatedly reactive to LG HCD 3.0 or DONG-A HCV 3.0 and further confirmed to be indeterminate by LG HCD CONFIRM at 14 Korean Red Cross Blood Centers in 1997. Correlation between HCV RNA and reactivity to each recombinant antigens was analysed. Three kinds of EIA kits such as HCD 3.0, DONG-A HCV 3.0 and Cobas Core Anti-HCV EIA kits were tested of their reactivity to 56 samples that had shown single band positivity on LG HCD CONFIRM. RESLUTS: HCV RNA was detected in 11 (6.1%) of 180 samples. Positivity of 897 band showed positive correlation with HCV RNA positivity (p<0.001), while Core518 showed negative correlation (p<0.001). DONG-A HCV 3.0 showed negative reaction to two samples with anti-897 specificity and HCV RNA, while LG HCD 3.0 and Cobas Core HCV showed negative reaction to one sample with anti-EIE2NS4 and HCV RNA. CONCLUSION: Positive rate of anti-HCV immunoblot indeterminate samples from Korean blood donors was 6.1%. In case of indeterminate results by LG HCD CONFIRM, positive reaction to 897 band highly suggests the presence of HCV RNA, while Core518 the absence of HCV RNA. It is highly recommended that specificity of Core518 antigen be increased in LG HCD 3.0 or LG HCD CONFIRM and sensitivity of recombinant antigen from the 3rd non-structural region of HCV genome be increased in DONG-A HCV 3.0.
Blood Donors* ; Genome ; Humans ; Immunoenzyme Techniques* ; Red Cross ; RNA* ; Sensitivity and Specificity

BACKGROUND: The outcome of hematopoietic stem cell transplantation (HSCT) for patients with severe aplastic anemia (SAA) in Seoul National University Hospital was analyzed retrospectively. METHODS: Between January, 1990 and March, 1999, 25 patients with SAA underwent HSCT. Their medical records were reviewed. Statistical analyses were done about survival and complication after HSCT. RESULTS: The median age of patients was 22 (range, 14~43) and male to female ratio was 18 : 7. Twenty two were HLA matched non- identical siblings. Three were one identical twin, one one-locus mismatched father and one HLA matched unrelated donor, respectively. Conditioning regimens were CY/TLI (cyclophosphamide, total lymphoid irradiation) for 18 patients, CY/ATG (CY, antithymocyte globulin) for 3, CY/ buffy (CY, unirradiated buffy- coat) for 2, CY/ ATG/TLI for 1, BU/CY (busulfan, CY) for 1. For prophylaxis of graft-versus-host disease (GVHD), cyclosporine and methotrexate were used in all patients except for identical twin. The median nucleated cell dose given to patients was 4.5x108/kg (range, 2.0~5.9). All evaluable patients achieved absolute neutrophil count of 500/microliter after median 17 days of HSCT (range, 12~27) and untransfused platelet count over 20,000/microliter after median 21 days of HSCT (range, 13~67). Six patients (24%, grade I : 3, II : 1, III : 1, IV : 1) developed acute GVHD and 8 (32%, limited : 4, extensive : 4) developed chronic GVHD. Hepatic venoocclusive disease (VOD) occurred in 2 patients (8%). Rejection occured in 4 patients (16 %), but among 22 allogeneic transplant recipients from HLA matched siblings, only one (5%) lost graft. After a median follow-up of 32 months (range 9~120 months), 5 year overall survival of all patients was 87%, and that of 22 allogeneic recipients from HLA matched sibling donors was 95%. Four patients (16%) died. Causes of death were VOD in one case, rejection with pneumonia one, acute GVHD one. One died from traffic accident in a cured state. CONCLUSION: Experiences from our center suggest that HSCT is an effective treatment for patients with severe aplastic anemia. Long- term survival is especially excellent for patients who have matched related donors.
Accidents, Traffic ; Anemia, Aplastic* ; Cause of Death ; Cyclosporine ; Fathers ; Female ; Follow-Up Studies ; Graft vs Host Disease ; Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells* ; Humans ; Male ; Medical Records ; Methotrexate ; Neutrophils ; Platelet Count ; Pneumonia ; Retrospective Studies ; Seoul ; Siblings ; Tissue Donors ; Transplantation ; Transplants ; Twins, Monozygotic ; Unrelated Donors

BACKGROUND: Acute myelogenous leukemia (AML) is the most common cause of leukemia in adults. Allogeneic bone marrow transplantation (BMT) for the treatment of AML is done worldwide now. METHODS: Between November 1987 and June 1998, we performed allogeneic BMT for 27 patients with AML from HLA-identical sibling donors. We reviewed medical records of these patients. RESULTS: The median age of patients was 31 (range, 15~43) and male to female ratio was 18 : 9. Conditioning regimens were BU/CY (busulfan, cyclophosphamide) for 22 patients, TBI/CY (total body irradiation, cyclophosphamide) for 3 patients, and TBI/VP/CY (TBI, VP-16, cyclophosphamide) for 2 patients. Cyclosporine and methotrexate were used in 18 patients for prophylaxis of graft-versus-host disease (GVHD), and cyclosporine and methyl-prednisolone were used in 9 patients. The median nucleated cell dose given to patients was 4.1x108 /kg. All evaluable patients achieved absolute neutrophil count of 500 /microliter after median 15 days after BMT (range, 11~45 days). Twenty-five percent of patients developed acute GVHD (> or = grade II) and there was no patient with grade IV acute GVHD. Twenty-nine percent developed chronic GVHD. Hepatic venoocclusive disease (VOD) occurred in 7 patients (26%). At the time of BMT, 16 patients were in the first remission status and 11 patients were in the advanced disease status. After a median follow-up of 27 months (range 7~127 months), the actuarial disease-free survival at 5 years was significantly higher in the first remission group than the others (44% vs. 9%; P=0.05). The difference of 5 year overall survival between these two groups approached statistical significance (50%for the first remission group and 12% for the others; P=0.13). There were 17 deaths. The causes of death were relapse (8 patients, 47%), VOD (3 patients, 18%), sepsis (2 patients, 12%), interstitial pneumonia (2 patients, 12%), chronic GVHD (1 patient, 6%), and drug-toxicity (1 patient, 6%). Eary deaths (<100 days) occurred in 6 patients (22%). CONCLUSION: Allogeneic BMT for patients with AML was most successful when done during the first remission. Clinical features of patients with AML treated with allogeneic BMT were similar to those from Western countries, but the incidence and severity of acute GVHD seem to be lower.
Adult ; Bone Marrow Transplantation* ; Bone Marrow* ; Cause of Death ; Cyclosporine ; Disease-Free Survival ; Etoposide ; Female ; Follow-Up Studies ; Graft vs Host Disease ; Humans ; Incidence ; Leukemia ; Leukemia, Myeloid, Acute* ; Lung Diseases, Interstitial ; Male ; Medical Records ; Methotrexate ; Neutrophils ; Recurrence ; Retrospective Studies* ; Sepsis ; Siblings ; Tissue Donors

BACKGROUND: To characterize the clinical features and determine the prognostic factors of severe community-acquired pneumonia. This study is the first of its kind in Korea. METHODS: Recruited were 40 patients diagnosed as severe community-acquired pneumonia in Hallym University Hospital from January 1, 1989 through July 31, 1996. Patients were analysed retrospectively for age, sex, underlying disease, respiration rate, hypoxemia, requirement of mechanical ventilation, involvement on chest radiograph, shock, and the serum concentration of BUN and albumin. All parameters were compared between survived and dead group. RESULTS: Male to female ratio was 2.07 : 1. The mean age was 63.1+/-l7.5years(range 25-90years) with 65% of patients aged equal to or more than 60. The major underlying diseases were old pulmonary tuberculosis(12.5%), chronic obstructive pulmonary disease(7.5%), bronchial asthma(5%), bronchiectasis(2.5%), and diabetes mellitus(22.5%). Microbiologic diagnosis was made in 26 out of 40 patients(65%). The most common causative organism was S. Pneumoniae(17.5%, 7/40) followed by S. aureus(15.0%, 6/40), K. Pncumoniac(12.5%, 5/40), M. tubercut osis(7.5%, 3/40), H. influenzae(2.5%, 1/40), coagulase negative staphylococcus(2.5%, 1/40), P aeruginosa(2.5%, 1/40), E. cloaceae(2.5%, 1/40), and E. coli(2.5%, 1/40). M. Pneumoniae was detected in no patient. The most frequent drugs administered in single or combination therapy were aminoglycosides(75%, 30/40), second- and third-generation cephalosporin(40%, 16/40 and 27.5%, 11/40), macro]ides(27.5%, 11/40), and amoxicillin/clavulanic acid(22.5%, 9/40). Of the 40 patients, 14 died of severe community-acquired pneumonia(37.S%). Among them, seven patients (50%) expired within 72h of hospital arrival. According to multivariate analysis, mortality was significantly associated with requirement of mechanical ventilation, bilateral pulmonary involvement, and serum albumin < or = 3.0g/dl. CONCLUSION: An understanding of the clinical charateristics and prognostic factors in severe community-acquired pneumonia identified in this study will optimize therapeutic approach in this disease and help decreasing its notorious mortality rate.
Anoxia ; Coagulase ; Diagnosis ; Female ; Humans ; Korea ; Male ; Mortality ; Multivariate Analysis ; Pneumonia* ; Prognosis ; Radiography, Thoracic ; Respiration, Artificial ; Respiratory Rate ; Retrospective Studies ; Serum Albumin ; Shock

Anti-thrombin III deficiency is known as a disease of autosomal dominant trait and relatively common, but in Korea, exact incidence and mortality is not known, In general, Anti-thrombin III deficiency is expressed to venous thromboembolism like deep vein thrombosis or pulmonary embolism. But, arterial embolism is very rare. We experienced a case of Antithrombin III deficiency expressed as myocardial infarction of inferior wall by huge thrombosis in the mid and distal right coronary artery.
Antithrombin III Deficiency ; Coronary Vessels ; Embolism ; Incidence ; Korea ; Mortality ; Myocardial Infarction* ; Pulmonary Embolism ; Thrombosis ; Venous Thromboembolism ; Venous Thrombosis

BACKGROUND: Uncomplicated myocardial infarction is often the harbinger of future cardiac events such as unstable angina pectoris,recurrent myocardial infarction or death. The feasibility and safety of exercise testing performed soon after myocardial infarction have been established but the prognostic value of exercise test after myocardial infarction remain inconclusive. The object of this study is to determine whether exercise test results can be utilized to predict of future cardiac events after uncomplicated myocardial infarction. METHODS: The study group comprised 149 patients with an uncomplicated myocradial infarction. A low level exercise test was performed before discharge from the hospital 8 to 10 days after myocardial infarction. The exercise thst results was considered positive if there was new > or =1mm horizontal or downsloping ST segment depression at 0.08 sec after the J point compared with baseline. The patients were followed for the development of new cardiac events. RESULTS: 1) The exercise test after acute myocardial infarction was performed in 149 patients without complication. The mean duration of exercise test was 14 min(range 1-20 min) and the mean work-load(Metabolic equivalents) was 3.7+/-1.1 METs. 2) 37 patients had ST-segment depression, 13 had ST-segment elevation and 27 had an inadequate blood pressure response to exercise. During the exercise, there were angina in 5 patients, dyspnea in 17 and no symptom in 127 patients. 3) During the follow-up period(1 to 75 month, mean 27.4 month), 29 patients experienced post-myocardial infarction angina, 1 had recurrent myocardial infarction, 4 had revascularization therapy(PTCA 2, CABG 2),5 had ischemic cardiomyopathy and 5 died a cardiac death. 4) The patients with cardiac events such as cardiac death, myocardial infarction and post MI angina had a significantly shorter exercise duration(13.1+/-4.0 and 14.6+/-2.7min, p<0.05), lower exercise tolerance(3.5+/-1.0 and 3.9+/-1.0 METs, p<0.05) and lower peak heart rate(117 +/- and 126+/-5, p<0.05). 5) The ST-segment depression, lower exercise tolerance(<3.0 METs) and history of hypertension were associated significantly with cardiac events(p<0.05) but ST-segment elevation, inadequate blood pressure response to exercise, the use of thrombolytic agents and non-Q wave infarction did not predict future cardiac events. Conclusions: The exercise test after acute myocardial infarction is safe and of limited value for predicting patients at risk of cardiac events in the follow-up period. The ST-segment depression and lower exercise tolerance(<3.0 METs) can predict cardiac events and the prognosis of the patients of this group can be improved with aggressive management and careful follow-up.
Angina, Unstable ; Blood Pressure ; Cardiomyopathies ; Death ; Depression ; Dyspnea ; Exercise Test* ; Fibrinolytic Agents ; Follow-Up Studies ; Heart ; Humans ; Hypertension ; Infarction ; Myocardial Infarction* ; Prognosis