BACKGROUND: Although the overall survival of childhood acute lymphoblastic leukemia (ALL) approaches 85-90%, the prognosis of relapsed or refractory (R/R) ALL is grave. This study aimed to identify the treatment pattern, treatment response, and overall survival of these patients.METHODS: We reviewed data of 64 patients with R/R ALL whose initial diagnosis of ALL had been made between 1 and 21 years of age. Patients who received clofarabine as part of an induction regimen were excluded. Relapsed patients were limited to those who relapsed after ≥2 prior induction regimens. Treatment patterns, response rates, and overall survival were analyzed.RESULTS: Patients' median age was 15.0 years (range, 6.0-25.0) at the diagnosis of R/R ALL. The most frequently used agents other than steroid were vincristine (54.0%), cytarabine (44.6%), and idarubicin (36.5%), while L-asparaginase was used in only one patient. The complete remission (CR) and overall response (OR) rates were 38.1 and 42.9%, respectively. Sixteen patients (25.4%) underwent allogeneic hematopoietic stem cell transplantation (HSCT). The 5-year overall survival was 6.7%. The survival of patients with HSCT was significantly higher compared with those without HSCT (35.2% vs 0%, P=0.0097). Among 14 patients who achieved CR or CR without platelet recovery (CRp) before HSCT, the 3-year survival was 46.9%.CONCLUSION: The survival of Korean patients with R/R childhood ALL was dismal despite a reasonable CR rate, whereas that of those who received HSCT after CR or CRp was excellent. More treatment options are needed to improve the overall outcome of R/R childhood ALL.
Blood Platelets ; Cytarabine ; Diagnosis ; Hematopoietic Stem Cell Transplantation ; Humans ; Idarubicin ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Prognosis ; Retrospective Studies ; Vincristine

This study was conducted to investigate long-term neurocognitive outcomes and to determine associated risk factors in a cohort of Korean survivors of childhood acute lymphoblastic leukemia (ALL). Forty-two survivors of ALL were compared with 42 healthy controls on measures of a neurocognitive test battery. We analysed potential risk factors (cranial irradiation, sex, age at diagnosis, elapsed time from diagnosis, and ALL risk group) on neurocognitive outcomes. ALL patients had lower, but non-significant full-scale intelligence quotient (FSIQ, 107.2 +/- 12.2 vs. 111.7 +/- 10.2), verbal intelligence quotient (VIQ, 107.7 +/- 13.6 vs. 112.2 +/- 11.4), and performance intelligence quotient (PIQ, 106.3 +/- 14.2 vs. 110.1 +/- 10.7) scores than healthy controls. However, patients treated with cranial irradiation performed significantly lower on FSIQ (102.2 +/- 8.1), VIQ (103.3 +/- 11.7), and PIQ (101.4 +/- 13.2) compared to non-irradiated patients and healthy controls. ALL patients also had poor attention, concentration, and executive functions. Among ALL survivors, cranial irradiation was a risk factor for poor FSIQ, being male was a risk factor for poor PIQ, and younger age was a risk factor for poor attention. Therefore, the delayed cognitive effects of ALL treatment and its impact on quality of life require continuing monitoring and management.
Adolescent ; Age Factors ; Child ; *Cognition ; Female ; Humans ; Intelligence ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/mortality/*psychology ; *Survivors ; Tertiary Healthcare

BACKGROUND: Diamond Blackfan anemia (DBA), characterized by impaired red cell production, is a rare condition that is usually symptomatic in early infancy. The purpose of this study was to assess nationwide experiences of DBA encountered over a period of 20 years. METHODS: The medical records of 56 patients diagnosed with DBA were retrospectively reviewed from November 1984 to July 2010. Fifteen institutions, including 13 university hospitals, participated in this study. RESULTS: The male-to-female ratio of patients with DBA was 1.67:1. The median age of diagnosis was 4 months, and 74.1% were diagnosed before 1 year of age. From 2000 to 2009, annual incidence was 6.6 cases per million. Excluding growth retardation, 38.2% showed congenital defects: thumb deformities, ptosis, coarctation of aorta, ventricular septal defect, strabismus, etc. The mean hemoglobin concentration was 5.1+/-1.9 g/dL, mean corpuscular volume was 93.4+/-11.6 fL, and mean number of reticulocytes was 19,700/mm3. The mean cellularity of bone marrow was 75%, with myeloid:erythroid ratio of 20.4:1. After remission, 48.9% of patients did not need further steroids. Five patients with DBA who received hematopoietic transplantation have survived. Cancer developed in 2 cases (3.6%). CONCLUSION: The incidence of DBA is similar to data already published, but our study had a male predilection. Although all patients responded to initial treatment with steroids, about half needed further steroids after remission. It is necessary to collect further data, including information regarding management pathways, from nationwide DBA registries, along with data on molecular analyses.
Anemia ; Anemia, Diamond-Blackfan ; Aortic Coarctation ; Bone Marrow ; Congenital Abnormalities ; Diamond ; Erythrocyte Indices ; Heart Septal Defects, Ventricular ; Hemoglobins ; Hospitals, University ; Humans ; Incidence ; Korea ; Male ; Medical Records ; Registries ; Reticulocytes ; Retrospective Studies ; Steroids ; Strabismus ; Thumb ; Transplants

BACKGROUND: The purpose of this study was to determine the spectrum of locally prevalent pathogens and their susceptibility patterns responsible for bacteremia in pediatric hemato-oncologic patients for empiric antimicrobial therapy. MATERIALS AND METHODS: A one-year retrospective study of pediatric hematooncologic patients with bacteremia in Seoul St. Mary's Hospital, the Catholic University of Korea, from April 2009 to March 2010 was conducted using previous medical records. The findings were compared with our previous data obtained from 2004 to 2006. RESULTS: Sixty-five episodes of bacteremia were recorded in 41 patients. Of them, 55 (84.6%) occurred in neutropenic and 10 (15.4%) in non-neutropenic patients. Gram-positive organisms were more commonly isolated than Gram-negative organisms (56.9% vs. 41.5%) in the following order: viridans streptococci (23.1%), Klebsiella pneumoniae (21.6%), coagulase-negative staphylococci (12.3%), Staphylococcus aureus (7.7%), Enterococcus faecium (7.7%). Susceptibility rates of viridans streptococci to penicillin, cefotaxime and vancomycin were 33.3%, 60% and 100%, and those of Enterobacteriaceae to amikacin, ceftazidime, piperacillin/ tazobactam and meropenem were 94.7%, 73.7%, 78.9%, and 100%, respectively. Compared to our previous data, infection still contributed towards a major fraction of mortality and morbidity in the management of patients with cancer. No differences in mortality rate were observed between isolated organisms from bacteremia. CONCLUSIONS: Gram-positive organisms were more prevalent than Gram-negative organisms in our population. The monitoring of causative agents and antimicrobial resistance should be considered in therapeutic strategies of pediatric hemato-oncologic infection.
Amikacin ; Bacteremia ; Cefotaxime ; Ceftazidime ; Child ; Enterobacteriaceae ; Enterococcus faecium ; Humans ; Klebsiella pneumoniae ; Korea ; Medical Records ; Neutropenia ; Penicillanic Acid ; Penicillins ; Retrospective Studies ; Staphylococcus aureus ; Thienamycins ; Vancomycin ; Viridans Streptococci

We investigated the outcome of idarubicin plus N4-behenoyl-1-beta-D-arabinofuranosyl cytosine (BHAC)-based chemotherapy (BHAC group, n=149) compared to idarubicin plus cytarabine-based chemotherapy (cytarabine group, n=191) for childhood acute myeloid leukemia (AML). Between January 1996 and December 2005, 340 children with AML from 5 university hospitals in Korea received the BHAC-based or cytarabine-based chemotherapy, with or without hematopoietic stem cell transplantation. After induction therapy, 264 (77.6%) of 340 children achieved a complete remission (CR) and 43 (12%) achieved a partial remission (PR). The CR rate in the BHAC group was higher than in the cytarabine group (85.2% vs. 71.7%, P=0.004). However, the overall response rate (CR+PR) was not different between the two groups (93.3% vs. 87.9%, P=0.139). The 5-yr estimates of overall survival (OS) of children in the two groups were similar (54.9% for the BHAC group vs. 52.4% for the cytarabine group, P=0.281). Although the results were analyzed according to the treatment type and cytogenetic risk, the OS showed no significant difference between the BHAC group and the cytarabine group. In the present study, the clinical outcomes of the BHAC-based chemotherapy, consisting of BHAC, idarubicin, and 6-TG, are comparable to that of the cytarabine-based chemotherapy for childhood AML.
Adolescent ; Antineoplastic Combined Chemotherapy Protocols/*therapeutic use ; Child ; Child, Preschool ; Combined Modality Therapy ; Cytarabine/*analogs & derivatives/*therapeutic use ; Cytogenetics ; Female ; Hematopoietic Stem Cell Transplantation ; Humans ; Idarubicin/*therapeutic use ; Infant ; Infant, Newborn ; Leukemia, Myeloid, Acute/*drug therapy/mortality ; Male ; Republic of Korea ; Retrospective Studies ; Survival Analysis ; Thioguanine/*therapeutic use ; Young Adult

The purpose of this study is to evaluate predictors of success of repeated injections of methotrexate in the single-dose regimen for the treatment of tubal ectopic pregnancy. All patients who had ectopic tubal pregnancy and were treated with a single dose regimen were retrospectively identified. 126 patients were treated with methotrexate. Among them, 39 patients were adequate for this study. 33 were treated with the 2nd dose and 27 were successfully cured. Additionally, 6 who were injected with the 3rd dose were all cured as well. Therefore, in our study, the success rate for the repeated injections of methotrexate was found to be 84.6% (33/39). The mean initial beta-hCG level was significantly lower in patients who were successfully treated than in patients who failed (3915.3+/-3281.3 vs. 8379.7+/-2604.4 IU/mL, p<0.05). The success rate is 96% when the beta-hCG level is less than 6,000 IU/mL and is 58% when beta-hCG is greater than 6,000 IU/mL (OR=18.57, 95% CI 1.86-185.89). The initial beta-hCG level is the only factor that has significant meaning as predictor of success of repeated injections of methotrexate in the single-dose regimen. Repeated injections of methotrexate may be particularly effective when the initial beta-hCG level is below 6,000 IU/mL.
Abortifacient Agents, Nonsteroidal/administration & dosage/therapeutic use ; Chorionic Gonadotropin, beta Subunit, Human/blood ; Female ; Humans ; Injections ; Methotrexate/administration & dosage/*therapeutic use ; Predictive Value of Tests ; Pregnancy ; Pregnancy, Tubal/blood/*drug therapy ; Retrospective Studies ; Time Factors ; Treatment Outcome

BACKGROUND: Angiotensin converting enzyme inhibitor or Angiotensin II type 1 receptor blocker is usually recommended for hypertensive patients with diabetes mellitus. No reports have been made on antihypertensive agents prescribed for these patients in private practice. METHODS: During a one month period of December 2004, 169 hypertensive patients with diabetes mellitus on their prescribed antihypertensive agents by ten family physicians were analyzed. The influencing factors for such prescriptions were analyzed by directly visiting each physicians who prescribed them. RESULTS: A total of 21 antihypertensive agents were prescribed by these family physicians. Single calcium channel blocker therapy was the most common with 55 cases (32.5%), followed by 20 cases (11.8%) of single angiotensin converting enzyme inhibitor therapy and 16 cases of single therapy of Angiotensin II type 1 receptor blocker. The low frequency of prescribing the Angiotensin II type 1 receptor blocker was due to insufficient effect (7), lack of information (5), resistance from the patients from changing the medications (4) and expensive costs (4). CONCLUSION: In prescribing antihypertensive agents for patients with diabetes mellitus, angiotensin converting enzyme inhibitor or Angiotensin II type 1 receptor blocker were chosen less. In order to decrease the incidence of complications in these patients, such agents should be prescribed more.
Antihypertensive Agents* ; Calcium Channels ; Diabetes Mellitus* ; Humans ; Hypertension ; Incidence ; Peptidyl-Dipeptidase A ; Physicians, Family ; Prescriptions ; Primary Health Care* ; Private Practice ; Receptor, Angiotensin, Type 1

PURPOSE: Children with Malignant lymphoma who is in the advanced stage at diagnosis or relapses during treatment have a poor prognosis. Recently, hematopoietic stem cell transplantation (HSCT) for advanced stage or refractory/relapsed lymphoma performed frequently. However, the role for HSCT for children with malignant lymphoma is still controversial. In this study, we reviewed children with malignant lymphoma who received HSCT and analyzed the results. METHODS: Questionnaires were made and sent to a group of teaching hospitals, with a return of 37 questionnaires from 11 hospitals. 33 patients with Non-Hodgkin lymphoma (NHL) and 4 patients with Hodgkin disease (HD) who received HSCT from 1997 to 2004 in Korea were enrolled in this study. Disease state at diagnosis, relapses during treatment, disease state at HSCT, and survival record were analyzed. All Data were reviewed with the questionnaires from the 11 teaching hospitals. RESULTS: Four patients with HD received HSCT at the 2nd complete remission after relapse. Survival rate for HD was 100% and their follow up duration ranged from 0.2 to 6.2 years (median 2.4 years). The 2-year survival rate for NHL was 68.1+/-9.0% and their follow up duration ranged from 0.1 to 7.6 years (median 1.5 years). The 2-year survival rate in patients with advanced stage at diagnosis and in relapsed/refractory patients were 83.6+/-1.1% and 55.9+/-12.9%, respectively (P=0.12). The mortality asssociated with HSCT was only 1 case, and most of the transplantation related complications did not resulted in death. CONCLUSION: Our results suggest that high dose chemotherapy followed by HSCT in children with malignant lymphoma is a safe procedure, which at the same time improves the results of standard treatment.
Child ; Diagnosis ; Drug Therapy ; Follow-Up Studies ; Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells* ; Hodgkin Disease ; Hospitals, Teaching ; Humans ; Korea* ; Lymphoma* ; Lymphoma, Non-Hodgkin ; Mortality ; Prognosis ; Surveys and Questionnaires ; Recurrence ; Retrospective Studies* ; Stem Cell Transplantation ; Survival Rate

OBJECTIVE: The goal of this clinical study was to evaluate clinical and pathological findings, surgical procedures, and postoperative treatment in women with ovarian granulosa cell tumor. METHODS: A clinical study was made on 28 cases of ovarian granulosa cell tumor that were treated at the department of Obstetrics and Gynecology in Korea University Hospital between January 1978 and December 2002. Data for 28 women with granulosa cell tumor were collected retrospectively. Follow-up data were collected from the hospital records or telephone survey. Fisher's exact test and Kaplan-Meier survival analysis were applied, as appropriate. RESULTS: The mean age of patients was 49 years (range, 28 to 71 years). The most common present symptom was vaginal bleeding (11/28, 39.3%). 82.6% of patients were with FIGO stage I, 3.6% with stage II, 7.1% with stage III, and none with stage IV. Follow-up time was 62 months (5-128 months). The five year disease free survival rate was 90.4% and 10 year disease free survival rate was 86.5%. Significant prognostic factors were FIGO stage and the rupture of tumor. CONCLUSION: Even though granulosa cell tumor usually has good prognosis, it is a tumor of unquestionable malignant potential and has a tendency for late relapse. Long term follow-up is recommended. Multicenter prospective randomized studies are needed to identify the more rational treatment strategies of these rare malignant tumors.
Disease-Free Survival ; Female ; Follow-Up Studies ; Granulosa Cell Tumor* ; Granulosa Cells* ; Gynecology ; Hospital Records ; Humans ; Korea ; Obstetrics ; Ovary ; Prognosis ; Recurrence ; Retrospective Studies ; Rupture ; Telephone ; Uterine Hemorrhage

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