Purpose: Acute promyelocytic leukemia (APL) is a rare disease in children and there are some different characteristics between children and adult. We aimed to evaluate incidence, clinical characteristics and treatment outcomes of pediatric APL in Korea. Materials and Methods: Seventy-nine pediatric APL patients diagnosed from January 2009 to December 2016 in 16 tertiary medical centers in Korea were reviewed retrospectively. Results: Of 801 acute myeloid leukemia children, 79 (9.9%) were diagnosed with APL. The median age at diagnosis was 10.6 years (range, 1.3 to 18.0). Male and female ratio was 1:0.93. Thirty patients (38.0%) had white blood cell (WBC) count greater than 10×109/L at diagnosis. All patients received induction therapy consisting of all-trans retinoic acid and chemotherapy. Five patients (6.6%) died during induction chemotherapy and 66 patients (86.8%) achieved complete remission (CR) after induction chemotherapy. The causes of death were three intracranial hemorrhage, one cerebral infarction, and one sepsis. Five patients (7.1%) suffered a relapse during or after maintenance chemotherapy. The estimated 4-year event-free survival and overall survival (OS) rates were 82.1%±4.4%, 89.7%±5.1%, respectively. The 4-year OS was significantly higher in patients with initial WBC < 10×109/L than in those with initial WBC ≥ 10×109/L (p=0.020). Conclusion This study showed that the CR rates and survival outcomes in Korean pediatric APL patients were relatively good. The initial WBC count was the most important prognostic factor and most causes of death were related to serious bleeding in the early stage of treatment.

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.

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors originating in the gastrointestinal tract. With the introduction of molecular-targeted therapy for GISTs which has yielded remarkable outcomes, these tumors have become a model of multidisciplinary oncological treatment. Although Western clinical guidelines are available for GISTs, such as those published by the National Comprehensive Cancer Network (NCCN) and the European Society of Medical Oncology (ESMO), the clinical situations in Asian countries are different from those in Western countries in terms of diagnostic methods, surgical approach, and availability of new targeted agents. Accordingly, we have reviewed current versions of several GIST guidelines published by Asian countries (Japan, Korea, China, and Taiwan) and the NCCN and ESMO and discussed the areas of dissensus. We here present the first version of the Asian GIST consensus guidelines that were prepared through a series of meetings involving multidisciplinary experts in the four countries. These guidelines provide an optimal approach to the diagnosis and management of GIST patients in Asian countries.
Asian Continental Ancestry Group* ; China ; Consensus* ; Diagnosis* ; Gastrointestinal Stromal Tumors* ; Gastrointestinal Tract ; Humans ; Imatinib Mesylate ; Korea ; Medical Oncology

Bilateral interstitial infiltration in chest radiography, which may be fine granular, reticular or of ground glass opacity, is the typical radiographic findings of Pneumocystis jiroveci pneumonia. Recently, atypical radiographic features, including cystic lung disease, spontaneous pneumothorax or nodular opacity, have been reported intermittently in patients with P. jiroveci pneumonia. We report the case of a 29-year-old woman with a transplanted kidney whose simple chest radiography and HRCT scan showed numerous miliary nodules in both lungs, mimicking miliary tuberculosis (TB). Under the presumptive diagnosis of miliary TB, empirical anti-TB medication was started. However, Grocott methenamine silver nitrate staining of a transbronchial lung biopsy tissue revealed P. jiroveci infection without evidence of TB. These findings suggest that even in TB-endemic area other etiology such as P. jiroveci as well as M. tuberculosis should be considered as an etiology of miliary lung nodules in immunocompromised patients.
Adult ; Biopsy ; Bronchoscopy ; Female ; Glass ; Humans ; Immunocompromised Host ; Kidney ; Kidney Transplantation ; Lung ; Lung Diseases ; Methenamine ; Pneumocystis ; Pneumocystis jirovecii ; Pneumonia ; Pneumothorax ; Thorax ; Transplants ; Tuberculosis ; Tuberculosis, Miliary

We have evaluated the efficacy and safety of cetuximab plus FOLFIRI for irinotecan and oxaliplatin-refractory colorectal cancers. From September 2004 to February 2006, 31 patients with metastatic colorectal cancer were treated with cetuximab (400 mg/m2 intravenously [IV] over 2 hr on day 1 followed by weekly 1-hr infusions of 250 mg/m2) plus bi-weekly FOLFIRI (irinotecan 150 mg/m2 IV over 90 min, and leucovorin 100 mg/m2 IV over 2 hr, followed by 5-FU 400 mg/m2 IV bolus on day 1, and followed by 5-FU 2,400 mg/m2 by continuous IV over 46 hrs). Patients received a median of four cycles (range: 1-23). Eight (25.8%) patients had confirmed partial responses and 10 (32.2%) had stable disease. After a median follow-up of 13.2 months for surviving patients, the median time to progression was 2.9 months, the median duration of response was 5.4 months, and the median overall survival was 10.9 months. Skin toxicity was observed in 25 patients (80.4%) including grade 3 in 6 patients (19.4%). Other common non-hematologic toxicities of all grades were mucositis (32.3%), asthenia (22.6%), diarrhea (12.9%), and paronychial cracking (12.9%). The combination of cetuximab with FOLFIRI was effective and tolerable in colorectal cancer patients heavily pretreated with a number of chemotherapy regimens.
Adult ; Aged ; Antibodies, Monoclonal/*administration & dosage/adverse effects ; Antineoplastic Agents/*administration & dosage/adverse effects ; Antineoplastic Combined Chemotherapy Protocols/*administration & dosage/adverse ; Camptothecin/administration & dosage/adverse effects/analogs & derivatives ; Colorectal Neoplasms/*drug therapy/secondary ; Drug Resistance, Neoplasm ; Female ; Fluorouracil/administration & dosage/adverse effects ; Humans ; Leucovorin/administration & dosage/adverse effects ; Male ; Middle Aged ; Organoplatinum Compounds/pharmacology ; Prognosis ; Receptor, Epidermal Growth Factor/antagonists & inhibitors ; Safety

PURPOSE: We investigated the effects of restricted fluid in the first 7 days of life on the risk of bronchopulmonary dysplasia (BPD) or patent ductus arteriosus (PDA) in very low birth weight (VLBW) infants. METHODS: Eighty three VLBW infants who lived more than 28 days were selected. The amount of daily maintenance fluid was determined by calculation of insensible water loss (IWL) and urine output (UO). Seventy to 80 percent of calculated amount was given to the ventilated infants. Subjects were grouped into low (<25th%), moderate (25-75th%), and high (>75th%) fluid groups for the first 24 hours, 3 days and 7 days. Chi square tests analyzed proportions of subjects with or without morbidities across fluid groups. Multivariate logistic regression was used to analyze the effect of fluid intake on BPD or PDA, controlling for factors that are significantly associated with BPD or PDA by univariate analysis. RESULTS: Rates of BPD and PDA were not significantly associated with fluid groups on each time period. The result was the same after controlling for factors that are significantly associated with BPD or PDA by univariate analysis. For the first 3 and 7 days, fluid intakes were positively related with maximal weight loss, urine output and mechanical ventilation duration. CONCLUSION: In VLBW infants, when given based on needs reflected from IWL and UO versus intake, relatively low fluid intakes in the first week of life do not decrease the risk of BPD or PDA, and vice versa. We suggest that calculation of daily fluid based on IWL and UO is appropriate for VLBW infants.
Bronchopulmonary Dysplasia* ; Ductus Arteriosus, Patent* ; Fluid Therapy ; Humans ; Infant* ; Infant, Newborn ; Infant, Very Low Birth Weight* ; Logistic Models ; Respiration, Artificial ; Water Loss, Insensible ; Weight Loss

BACKGROUND: Urine/serum protein electrophoresis (PEP) and immunofixation electrophoresis (IEP) for monoclonal protein (M-protein) are used for initial evaluation in patients with multiple myeloma. We evaluated the prognostic significance of M-proteinuria status and its association with other prognostic factors. METHODS: Between December 2002 and December 2004, 64 de novo symptomatic multiple myeloma patients with intact immunoglobulin (Ig) type were divided into two groups according to their initial urine PEP/IEP findings. RESULTS: Twenty-seven patients with undetectable or free light-chains only were classified into F group, and 37 with whole Ig with or without light-chains were classified into W group. The two groups were similar in sex, age, performance, azotemia, beta2-microglobulin, stage and treatment, but M-protein concentration was significantly higher in the W than in F group (5.1 vs 1.3g/dL, P<0.01). The overall response rate was significantly higher in F group than in W group (80.8% vs 63.6%, P=0.02), whereas the 2-year OS rate did not differ significantly between the groups (81.0% vs 57.7%, P=0.15). CONCLUSION: Monoclonal proteinuria is helpful in identifying patients with advanced disease and poorer prognosis in multiple myeloma.
Azotemia ; Electrophoresis ; Humans ; Immunoglobulins* ; Multiple Myeloma* ; Prognosis ; Proteinuria*

PURPOSE: Multifactorial in pathogenesis, bronchopulmonary dysplasia is difficult to predict based on any single factor, especially early in life. We evaluated clinical and ventilatory parameters in the first week of life, and their combinations were tested for early prediction of BPD. METHODS: Eighty-one very low birth weight (VLBW) infants born at gestational ages<32 weeks and mechanically ventilated for at least first 7 days were selected and classified into BPD (n=48, radiological findings and oxygen dependency at postconceptional age of 36 weeks) and non-BPD (n=33) groups. Clinical and ventilatory parameters on days 1, 4 and 7 were analyzed. Chi-square and t-test were used to compare individual variables between two groups. Multiple logistic regression analysis was done to identify risk factors for BPD. RESULTS: The mean gestational age and birth weight were lower in BPD group. In multivariate analysis, significant risk factors that predict BPD were gestational age<30 weeks (OR 0.112, 95% CI 0.016-0.767), maximum MAP > or =4.5 on day 7 (OR 3.982, 95% CI 1.046-15.162) and maximum FiO2> or =0.3 on day 7 (OR 7.626, 95% CI 1.570-37.054). The combination of these factors for prediction of BPD had a 79% positive predictive value with an 85% sensitivity. CONCLUSION: A number of clinical and ventilatory parameters in combination (gestation, maximum MAP and FiO2 on day 7) can predict BPD in VLBW infants early in life with a relatively high sensitivity and positive predictiveness. With early identification of infants prone to BPD, the clinicians may resort to more active measures to minimize lung injury and to prevent BPD.
Birth Weight ; Bronchopulmonary Dysplasia* ; Gestational Age ; Health Resorts ; Humans ; Infant* ; Infant, Newborn ; Infant, Very Low Birth Weight* ; Logistic Models ; Lung Injury ; Multivariate Analysis ; Oxygen ; Respiration, Artificial* ; Risk Factors

PURPOSE: Suprapubic bladder aspiration(SBA) is a widely accepted method of obtaining sterile urine for culture in neonates but has a relatively low success rate and a few serious complications during SBA procedure. To improve the chance of obtaining urine, we determined the ultrasonographic bladder parameters(diameters and volume) to confirm that the bladder is adequately distended for successful aspiration of urine. METHODS: In 94 newborn infants who required sterile collection of urine, ultrasonographic examination and SBA procedure were carried out. On the patient's suprapubic area, the maximal cephalocaudal and anteroposterior diameters were measured by sagittal scanning, and the maximal anteroposterior and transverse diameters by transverse scanning. Bladder volume was calculated using the formula for elliptic volume, and then urine was aspirated to maximum of 10 mL and the amount of urine aspirated was recorded. RESULTS: Sufficient urine for culture(>1 mL) was obtained by SBA in 86(91.5%) of 94 infants. In the sagittal view, when the cephalocaudal diameter of the bladder was greater than 20 mm and the anteroposterior diameter was greater than 15 mm, the success rate was 100%. The cephalocaudal diameter of the bladder in sagittal view showed a more significant positive correlation with aspirated urine amount(r=0.65, P<0.001). CONCLUSION: The ultrasonographic sagittal view improved the success rate of SBA in neonates. A SBA was more likely to be successful when the cephalocaudal diameter of the bladder was above 20 mm and the anteroposterior diameter was above 15 mm.
Humans ; Infant ; Infant, Newborn ; Ultrasonography ; Urinary Bladder* ; Urinary Tract Infections