Purpose: Effectiveness and safety of clofarabine (one of the treatment mainstays in pediatric patients with relapsed/refractory acute lymphoblastic leukemia [ALL]) was assessed in Korean pediatric patients with ALL to facilitate conditional coverage with evidence development. Materials and Methods: In this multicenter, prospective, observational study, patients receiving clofarabine as mono/combination therapy were followed up every 4-6 weeks for 6 months or until hematopoietic stem cell transplantation (HSCT). Response rates, survival outcomes, and adverse events were assessed. Results: Sixty patients (2-26 years old; 65% B-cell ALL, received prior ≥ 2 regimen, 68.3% refractory to previous regimen) were enrolled and treated with at least one dose of clofarabine; of whom 26 (43.3%) completed 6 months of follow-up after the last dose of clofarabine. Fifty-eight patients (96.7%) received clofarabine combination therapy. Overall remission rate (complete remission [CR] or CR without platelet recovery [CRp]) was 45.0% (27/60; 95% confidence interval [CI], 32.4 to 57.6) and the overall response rate (CR, CRp, or partial remission [PR]) was 46.7% (28/60; 95% CI, 34.0 to 59.3), with 11 (18.3%), 16 (26.7%), and one (1.7%) patients achieving CR, CRp, and PR, respectively. The median time to remission was 5.1 weeks (95% CI, 4.7 to 6.1). Median duration of remission was 16.6 weeks (range, 2.0 to 167.6 weeks). Sixteen patients (26.7%) proceeded to HSCT. There were 24 deaths; 14 due to treatment-emergent adverse events. Conclusion Remission with clofarabine was observed in approximately half of the study patients who had overall expected safety profile; however, there was no favorable long-term survival outcome in this study.

Sarcoidosis is a somewhat common pulmonary disease, but the concurrence of lung cancer and sarcoidosis in the same patient is very rare. Because sarcoidosis usually presents as mediastinal lymphadenopathies, this concurrence in a lung cancer patient detected radiologically is apt to be misunderstood to be mediastinal metastases, and it is thus considered to be an unresectable disease. We report a case of lung cancer associated with sarcoidosis that developed in a 65-year-old woman who underwent surgery. Radiological studies revealed a 1.9x1.7 cm mass in the left upper lobe with multiple enlarged bilateral mediastinal lymph nodes (2R, 3a, 4R, 4L, 5, 6, 7, 8R). Pathologic findings showed that the mass was a well-differentiated adenocarcinoma and all of the enlarged mediastinal lymph nodes were granulomas without cancer metastasis. We report this case with a review of the literature.
Adenocarcinoma ; Aged ; Dimaprit ; Female ; Granuloma ; Humans ; Lung ; Lung Diseases ; Lung Neoplasms ; Lymph Nodes ; Neoplasm Metastasis ; Sarcoidosis

Sarcoidosis is a somewhat common pulmonary disease, but the concurrence of lung cancer and sarcoidosis in the same patient is very rare. Because sarcoidosis usually presents as mediastinal lymphadenopathies, this concurrence in a lung cancer patient detected radiologically is apt to be misunderstood to be mediastinal metastases, and it is thus considered to be an unresectable disease. We report a case of lung cancer associated with sarcoidosis that developed in a 65-year-old woman who underwent surgery. Radiological studies revealed a 1.9x1.7 cm mass in the left upper lobe with multiple enlarged bilateral mediastinal lymph nodes (2R, 3a, 4R, 4L, 5, 6, 7, 8R). Pathologic findings showed that the mass was a well-differentiated adenocarcinoma and all of the enlarged mediastinal lymph nodes were granulomas without cancer metastasis. We report this case with a review of the literature.
Adenocarcinoma ; Aged ; Dimaprit ; Female ; Granuloma ; Humans ; Lung ; Lung Diseases ; Lung Neoplasms ; Lymph Nodes ; Neoplasm Metastasis ; Sarcoidosis

PURPOSE: Biliary complication (BC) is known as the most common and intractable complication after adult living donor liver transplantation (LDLT), but there is lack of large-volume studies with long-term follow-up. To assess the patterns of BC and their treatment results in adult recipients of LDLT. METHODS: 182 adult patients who received 156 right and 26 left liver grafts from January 2001 to December 2002 were selected after exclusion of dual-graft LDLT and short-term survivors. Methods of biliary reconstruction, types of BC, and treatment results of BC were analyzed. RESULTS: The median follow-up period was 38 months. Biliary reconstruction was done as single duct-to-duct anastomosis (DD, n=109), double DD (n=22), single hepaticojejunostomy (HJ, n=31), double HJ (n=16), and combination of DD and HJ (n=4). Overall patient or graft survival rate was 96.2% at 1 year and 93.3% at 3 years. BC-free survival rate was 83.4% at 1 year and 76.5% at 3 years. BC occurred much more often in right liver grafts. There were no statistical differences of BC between DD and HJ groups, and between single and double anastomoses groups. Most of anastomotic leak occurred during the first 1 month, but anastomotic stenosis occurred till 3 years. Small right graft duct around 3 mm in diameter became a significant risk factor of BC. Anastomotic leak occurred in 8 recipients, and 7 recovered after radiological, endoscopic, and surgical treatments. Anastomotic stenosis occurred in 34, and most of them were resolved by radiological intervention. CONCLUSION: The incidence of early BC could be reduced to below 10% by technical refinements, but additional late BC occurred till 3 years. Most of BC were successfully controlled by endoscopic and radiological treatments. DD seems to be avoided in small graft duct around 3 mm in diameter. Close surveillance for BC seems to be mandatory for the first 3 years.
Adult ; Anastomotic Leak ; Constriction, Pathologic ; Follow-Up Studies ; Graft Survival ; Humans ; Incidence ; Liver Transplantation* ; Liver* ; Living Donors* ; Risk Factors ; Survival Rate ; Survivors ; Transplants

PURPOSE: Wilms tumor is the most common malignant renal tumor in children. We investigated the epidemiology, clinical features and treatment outcome of the children with Wilms tumor in Korea during the recent 10 years. METHODS: Two hundred forty six patients were enrolled between January 1991 and December 2000 from 26 major hospitals in Korea. The data regarding the clinical features including sex, age, pathologic type, prognostic factor and treatment outcome of patients were analyzed retrospectively by review of patient's medical records. Kaplan-Meier survival curves were constructed, The differences between groups were analyzed by log-rank test. RESULTS: There were 130 males and 116 females. The incidence between the age of 1~4 years was the highest with 66.2%. The annual incidence rate per 1, 000, 000 population varied from 1.9 to 2.1. The 10 years overall survival rate according to sex, clinical stage, pathologic type and relapse were as follows: 88.6% in male, 90.9% in female, 100% in stage I, 94.7% in stage II, 92.1% in stage III, 63.4% in stage IV, 85.7% in stage V, 95.3% in favorable histology, 64.1% in unfavorable histology, 94.8% in non-relapse, and 40.9% in relapse. The relapse rate was 12%. The 10 years overall survival rate of 246 patients were 89.1%. CONCLUSION: Our results could provide the most recent and important clinical information on Wilms tumor of children in Korea.
Child ; Epidemiology* ; Female ; Humans ; Incidence ; Kaplan-Meier Estimate ; Korea* ; Male ; Medical Records ; Recurrence ; Retrospective Studies ; Survival Rate ; Treatment Outcome ; Wilms Tumor*

PURPOSE: Malignant lymphoma is the primary malignant tumor derived from lymphoid organs. It is composed of Hodgkin's disease and non-Hodgkin lymphoma. Recently, survival rate is on the rise due to improved combination chemotherapy, radiotherapy and high dose chemotherapy followed by hematopoietic stem cell transplantation. In South Korea, no epidemiologic studies concerning malignant lymphoma in the pediatric age group has been performed. Therefore, the Korean Society of Pediatric Hematology-Oncology retrospectively analyzed the incidence, pathologic subtypes, treatment strategies, and survival rates of pediatric malignant lymphomas in South Korea. METHOD: Questionnaires were made and sent to a group of training hospitals, with a return of 580 questionnaires from 24 hospitals. Among them, 517 reports were suitable for analysis. RESULTS: Among the 517 cases, Hodgkin's disease accounted for 58 cases and non-Hodgkin's lymphoma for 459 cases. Male to female ratio for malignant lymphoma was 2.7. Mean age at diagnosis was 8.3 years. Among the pathologic subtypes, mixed cellularity was the most frequent subtype for Hodgkin's disease. Most (70.7%) cases of non-Hodgkins lymphoma belonged to high grade NHL. Burkitt lymphoma accounted for 102 cases, and lymphoblastic lymphoma was found in 58 cases. Peripheral lymphadenopathy was the most common presenting sign upon diagnosis. B symptoms were significantly more frequent in Hodgkin's disease patients than in non-Hodgkin lymphoma patients. The Complete response rate was 62.1% for non-Hodgkin's lymphoma, and 82.8% for Hodgkin's disease. Overall 5 year survival rate was 60.0% in non-hodgkin's lymphoma, and 84.8% in Hodgkin's disease. CONCLUSION: The annual incidence of malignant lymphoma in Korea is 4.7 per million. In cases of chemotherapy-sensitive, refractory or relapsed malinant lymphoma, high dose chemotherapy followed by hematopoietic stem cell transplantation is vital for improved survival. For more systematic analysis of epidemiology on malignant lymphomas, better surveillance mechanisms on the occurrence of malignant lymphomas are crucial, and establishment of standardized treatment protocol for malignant lymphoma is required.
Burkitt Lymphoma ; Child* ; Clinical Protocols ; Diagnosis ; Drug Therapy ; Drug Therapy, Combination ; Epidemiologic Studies ; Epidemiology* ; Female ; Hematopoietic Stem Cell Transplantation ; Hodgkin Disease ; Humans ; Incidence ; Korea* ; Lymphatic Diseases ; Lymphoma* ; Lymphoma, Non-Hodgkin ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Surveys and Questionnaires ; Radiotherapy ; Retrospective Studies* ; Survival Rate

PURPOSE: The annual incidence of aplastic anemia (AA) in Asian countries is higher than in Western countries. The pathogenesis in AA has been investigated in hematopoiesis and immunology. Recently, the survival rate and the quality of life of the patients with AA have been steadily improved by the development of a variety of treatments such as the immunosuppressive therapy (IST), and hematopoietic stem cell transplantation (HSCT). The Korean Society of Pediatric Hematology-oncology retrospectively investigated the incidence, treatment strategies, survival rate, and time to become independent from transfusion in patients with AA, who were diagnosed from January 1st, 1991 to December 31st, 2000 in Korea. METHODS: All the questionnaires were sent to a group of training hospitals, and we collected about 600 questionnaire forms from 27 hospitals. However, 493 reports were available for data analysis. RESULTS: The male and female ratio in AA is 1.1 (259 males vs. 234 female). The median age at diagnosis is 9 years old (range; 0.8~16 years old). The annual incidence of children with AA in Korea is 4.5 per million on the basis of Korean pediatric population. In etiology, there are 20 cases (4.1%) in congenital and others in acquired AA. In acquired AA, the cause of illness was not identifiable for most of the patients, but 1 patient had hepatitis-associated AA, and 3 patients developed the illness after medication. According to the initial laboratory data at diagnosis, the peripheral blood findings showed that hemoglobin is 7.1+/-2.4 g/dL, white blood cell 3, 200/microL (200~16, 550), absolute neutrophil counts 670/microL (0~12, 487), platelets 19, 000/microL (1, 000~500, 000), and corrected reticulocytes 0.18% (0.0~4.7). The bone marrow examination revealed that cellularity was below 25% in 348 patients, and over 25% in 105 patients. In the available data, 269 patients (54.6%) were diagnosed of severe aplastic anemia (SAA) and 224 patients of non-SAA (NSAA). HSCT were done for 96 patients (19.5%) and others received another treatments such as the IST. The anti-thymocyte globulin (ATG) or anti-lymphocyte globulin (ALG) treatment was done for 263 cases, corticosteroids for 259, cyclosporine A (CSA) for 215, and anabolic steroids for 138. The combination IST including ATG or ALG plus corticosteroid plus CSA were applied to 154 children with AA, and transfusion only in 37, as conservative care. In case of those patients with HSCT, the time from diagnosis to transplantation was 12 months (1~144 months) and the sources of stem cells were bone marrow in 82 cases, growth factor mobilized peripheral blood in five, and cord blood in six. There were 57 patients transfused below 40 units of blood products before HSC transplantation. Graft rejection was identified from 16 patients, and booster transplantations were done for 12 patients among them. In complications of HSCT, the graft versus host disease was developed in 20 patients and viral diseases in 12 cases including the CMV, herpetic infection, and hepatitis. Also, one patient suffered from veno-occlusive disease. The overall survival rate in children with AA is 64.3%. The survival rate in HSCT is better than that IST (76.9% vs. 62.6%, P< 0.05). In IST, overall survival rate in very SAA showed lower than SAA and NSAA, and in SAA lower than NSAA in case of absolute neutrophil count below 200/microL (P< 0.05). There was no significant difference in terms of the sex, age at beginning of treatment. In HSCT, transfusion was not related to the survival rate. However, overall survival rate is better in short interval between diagnosis and HSCT than in long interval (P< 0.05). There was no significant difference in the probability of transfusion independence according to treatment strategies, even though it was 71.0% in HSCT and 12.8% in immunosuppressive therapy at the end point of survey (P=0.47). The response pattern was as follows. There were 155 cases of complete response, 110 of partial response and 120 of no response in spite of various treatments. The relapse after treatment was found in 11 patients after IST, of which 6 patients experienced more than 2nd relapse. The median time between the end of treatment and relapse was 16 months (6~84 months). Only three cases developed into other diseases (1 case into acute myeloid leukemia and 2 cases into myelodysplastic syndrome). The median time from diagnosis to the end of treatment was 62 months (0.5~174 months). In fatal cases, the median time between diagnosis and death was 29 months (0~144 months) despite several therapeutic strategies. CONCLUSION: In Korea, the annual incidence of children with AA is 4.5 per million. This result is similar to the ones reported in other Asian countries, but higher than those in Western countries. Although a lot of children with AA received various therapies including IST or HSCT, new treatment strategies have to be developed to improve the survival rate and the quality of life of children with AA
Adrenal Cortex Hormones ; Allergy and Immunology ; Anemia, Aplastic* ; Antilymphocyte Serum ; Asian Continental Ancestry Group ; Bone Marrow ; Bone Marrow Examination ; Child* ; Cyclosporine ; Diagnosis ; Epidemiology* ; Female ; Fetal Blood ; Graft Rejection ; Graft vs Host Disease ; Hematopoiesis ; Hematopoietic Stem Cell Transplantation ; Hepatitis ; Humans ; Incidence ; Korea* ; Leukemia, Myeloid, Acute ; Leukocytes ; Male ; Neutrophils ; Quality of Life ; Surveys and Questionnaires ; Recurrence ; Reticulocytes ; Retrospective Studies* ; Statistics as Topic ; Stem Cells ; Steroids ; Survival Rate ; Virus Diseases

BACKGROUND: Thoracoscopic bullectomy (VATS-B) is now the preferred treatment for spontaneous pneumothorax despite of higher recurrence rate than open thoracotomy. Several methods have been used to prevent this problem. The effectiveness of staple line reinforcement (SLR) in VATA-B using endostaplers was assessed by clinical and experimental study. MATERIAL AND METHOD: In experimental study, canine lungs were harvested immediately (group I, N=5) and 48 hours (group II, N=5) after stapling. The pressures at which initial air leaks occurred were measured. In clinical study from February 1997 to March 1999, 106 procedures in 104 patients undergoing VATS-B for spontaneous pneumothorax were classified into two groups according to the presence of SLR and were compared. RESULT: The average pressure of the initial air leakage was significantly higher in SLR than that of staples alone (18+/-1.6 vs 48+/-3 mm Hg in group I; 23.8+/-1.9 vs 54+/-4.6 mm Hg in group II, p<0.001). In the clinical data, there were significant differences seen in the duration of drainage, the total length of endostaplers used, and the duration of the postoperative hospital stay between patients with staple alone and patients with SLR (4.4+/-1.4 vs 3.1+/-1.1 days in duration of drainage, 92.3+/-28.1 vs 71.1+/-30.6 mm in total length of endostaplers used, 5.9+/-1.9 vs 4.6+/-1.7 days in postoperative hospital stays, p<0.001). CONCLUSION: SLR was effective for preventing prolonged air leakage and responsible for shorter hospital stays after VATS-B for the treatment of spontaneous pneumothorax.
Drainage ; Humans ; Length of Stay ; Lung ; Models, Theoretical ; Pneumothorax* ; Polytetrafluoroethylene* ; Recurrence ; Surgical Staplers ; Thoracoscopy ; Thoracotomy

PURPOSE: Recently Wilms tumor gene (WT1) transcripts have been detected in leukemia regardless of the disease subtype and the specific DNA markers suggesting that WT1 gene might be a useful panleukemic marker for monitoring minimal residual disease (MRD). This study was performed to investigate the expression of WT1 gene by a quantitative methods and to find the prognostic value of WT1 gene in childhood acute leukemia. METHODS: From the mononuclear cells isolated from bone marrow aspirates and peripheral bloods of 22 childhood acute and chronic leukemia patients, mRNA were extracted for the reverse transcriptase-polymerase chain reactions (RT-PCR). Relative levels of WT1 gene expression was calculated by using the value in K562 cell line to be 1.00 as a positive control. RESULTS: The sensitivity of detection of MRD with WT1 primers was 10 4 and comparable to that of bcr/abl expression in K562 cells and a patient with CML in blast crisis. WT1 gene expression was detected in 17 of 22 (77%) patients; 9/10 of acute lymphoblastic leukemia (ALL), 6/10 acute myelogenous leukemia (AML), 1 acute mixed lineage leukemia (AMLL) and 1 chronic myelogenous leukemia (CML) in blast crisis. In 4 AML patients who received autologous peripheral blood stem cell transplantation (PBSCT), two patients relapsed after reappearance of WT1 gene expression in bone marrow aspirates and the remaining two were in complete remission without expression of WT1 gene. CONCLUSION: These results show that WT1 gene expression is frequently noted in childhood acute leukemia and can be a useful sensitive marker for the detection of MRD comparable to bcr/abl transcripts. WT1 gene can be used as a panleukemic marker for the MRD monitoring for the evaluation of the remission status and in predicting early relapse in children with acute leukemia in the molecular levels. It may also be a useful tool for the detection of leukemic cell contamination in the process of peripheral blood stem cell transplantation.
Blast Crisis ; Bone Marrow ; Cell Line ; Child ; Gene Expression ; Genetic Markers ; Humans ; K562 Cells ; Leukemia* ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Leukemia, Myeloid, Acute ; Neoplasm, Residual ; Peripheral Blood Stem Cell Transplantation ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Recurrence ; RNA, Messenger ; Wilms Tumor

PURPOSE: To compare the differential findings of tuberculous otitis media(TOM) with those of chronic suppurative otitis media with or without cholesteatoma, as seen on high resolution temporal bone CT. MATERIALS AND METHODS: We retrospectively reviewed 14 cases of TOM, 30 cases of chronic suppurative otitis media(CSOM), and 30cases of chronic otitis media with cholesteatoma(Chole). All had been pathologically confirmed. We evaluated thepreservation of mastoid cells without sclerotic change, the location and extension of soft tissue to the externalauditary canal, the erosion of ossicles, the tegmen tympani, scutum, bony labyr inth, facial nerve canal andsigmoid sinus, and the presence of intracranial co mplications. RESULTS: Soft tissue in the mastoid antrum wasseen in all cases of TOM(100%), 29 cases of CSOM(96.7%), and 26 cases of Chole(86.7%). In contrast, the softtissue in the entire middle ear cavity was noted in 13 cases of TOM(92.8%), 7 cases of CSOM(23.3%), and 12 casesof Chole(40%). Soft tissue extended to the superior aspect of the external auditory canal in 4 cases of TOM(28.6%) and 5 cases of Chole (16.7%). Mastoid air cells were seen in 9 cases of TOM (64.3%), 4 cases of CSOM(13.3%), and 3 cases of Chole(10%). Ossicular erosion was noted in 6 cases of TOM (42.9%), 12 cases of CSOM (40%),and 26 cases of Chole(86.7%), while in one case of TOM (7.1%), 5 cases of CSOM (16.7%), and 15 cases of Chole(50%)there was erosion of the scutum. In one case of TOM, follow-up CT study after 9 months of antituberculousmedication without surgery revealed complete clearing of previously noted soft tissue in the middle ear cavity. CONCLUSION: Specific CT findings of TOM were not seen, but if there were findings of soft tissue in the entiremiddle ear cavity, soft tissue extension to the external auditory canal, preservation of mastoid air cells withoutsclerotic change, and intact scutum, TOM may be differentiated from other chronic otitis media.
Cholesteatoma ; Ear ; Ear Canal ; Ear, Middle ; Facial Nerve ; Follow-Up Studies ; Mastoid ; Otitis Media* ; Otitis Media, Suppurative ; Otitis* ; Retrospective Studies ; Temporal Bone* ; Tuberculosis