No abstract available.
Korea*

No Abstract Available.
Humans

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

BACKGROUND: Hematopoietic stem cell transplantation (HSCT) is one of the most important armamentarium against various hematologic malignancies or some solid tumors. We investigated the number of patients who might need transplants and compared with that of actual transplants to conceptualize current status and circumstances of HSCTs in Korean children. METHODS: Questionnaires were sent to Korean Society of Hematopoietic Stem Cell Transplantation (KSHSCT) members who were taking care of children with malignancies or hematologic diseases. Almost all of the newly diagnosed patients between Jan, 1st and Dec, 31st, 2003 were enrolled in the study. RESULTS: Seven hundred forty eight children (male to female ratio = 1.4:1) were enrolled. The median age was 6.1 years old (8 days~28.8 years old). Malignant diseases consisted of 695 cases (92.9%), and among them almost half were hematologic malignancies. The participating members speculated that HSCTs should be indicated in 285 children (38.1%) which included 209 allogeneic, and 76 autologous transplants. In reality, however, allogeneic HSCTs were performed only in 140 children (67.0%) with the median interval of 5.9 month, and autologous transplants in 44 children (57.9%) with 8.3 month. In autologous setting, all the patients received peripheral blood stem cells (PBSCs), whereas bone marrow (61%), cord blood (34%), and PBSC (5%) were used in allogeneic HSCTs. Donor types were as follows: unrelated donor (37%), cord blood (34%), sibling donor (25%), and family (4%). The reasons for not performing HSCTs were unfavorable disease status or death, no availability of suitable donor, economical situation, and refusal by parental preferences. Under the strict insurance regulations, many transplants were not covered by insurance. More autologous transplants were performed without insurance coverage than allogeneic HSCTs (P=0.013). Those cases were advanced cases and HLA mismatch transplants for allogeneic setting, and relatively rare diseases still awaiting favorable results of transplants for autologous setting. CONCLUSION: HSCTs are essential part of treatment strategies for children with various diseases. Unfortunately, however, a third of patients who were in need of transplants did not receive HSCTs due to various reasons. It is necessary to expand unrelated donor pool or cord blood banks for the cases lacking HLA-identical sibling donors. Also medical insurances should cover HSCTs for rare diseases as well as for less favorable but novel situations where there are no suitable alternatives.
Autografts ; Bone Marrow ; Child* ; Disulfiram ; Female ; Fetal Blood ; Hematologic Diseases ; Hematologic Neoplasms ; Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells* ; Humans ; Insurance ; Insurance Coverage ; Parents ; Rare Diseases ; Siblings ; Social Control, Formal ; Stem Cells ; Tissue Donors ; Unrelated Donors ; Surveys and Questionnaires

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: Acute lymphoblastic leukemia (ALL) accounts for approximately 75% of all cases of childhood leukemia. We investigated epidemiology, clinical and laboratory features and treatment outcome of the children with ALL in Korea during recent 5 years. METHODS: One thousand forty nine patients were enrolled between January 1994 and December 1998 from 37 major hospitals in Korea. The data regarding the clinical and laboratory features including age, WBC counts at diagnosis, immunophenotype, morphology, cytogenetics 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 analyzed by log-rank test. RESULTS: There were 597 males and 452 females. The distribution between the age 2 and 5 years is most common in 46.1%. The annual incidence rate per 100,000 population varied from 1.6 to 2.2. The 5 year event free survival (EFS) rates according to good prognostic factors were as follows: 67% bet ween 1-9 year of age at diagnosis, 69% in under 10,000/mm3of initial WBC count, 74% in early pre-B cell CALLA ( ) immunophenotype, 65% in L3 morphology, 68% in no CNS invasion. Most of patients were treated by CCG treatment protocol. The 5 year EFS was 63%. Main complications were sepsis (21.8%) and hemorrhage (12.5%). The relapse rate was 15.6%. The common causes of death were sepsis, DIC, pneumonia, relapse. CONCLUSION: Our results could provide the most recent and important information about acute lymphoblastic leukemia of children in Korea.
Cause of Death ; Child ; Clinical Protocols ; Cytogenetics ; Dacarbazine ; Diagnosis ; Disease-Free Survival ; Epidemiology* ; Female ; Hemorrhage ; Humans ; Incidence ; Kaplan-Meier Estimate ; Korea* ; Leukemia ; Male ; Medical Records ; Pneumonia ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Precursor Cells, B-Lymphoid ; Recurrence ; Retrospective Studies ; Sepsis ; Treatment Outcome