No abstract available.
Humans ; Transcription Factors*

Abnormalities of the short arm of chromosome 12 are relatively common in childhood ALL. Approximately 5~7% of children with ALL have cytogenetic evidence of a translocation involving 12p, whereas 3~5% have deletions which suggest the presence of a tumor suppressor gene at this location. Through the use of sensitive molecular techniques, 12p or 12p 12~13 loss of heterozygosity (LOH) has been demonstrated in approximately 25 oyo of childhood ALL cases. FISH mapping has detected a minimum region of overlap for the 12p deletions between the TEL(ETV6) and CDKN1B(KIP1) geness). Recently, chromosomal translocations involving the TEL gene at 12p13 have been cloned in several hematopoietic disorders. In ALL cells with thet(12;21)(p13;q22), the 5' part of TEL is fused with the AML1 gene. Abnormalities of 12p, especially the t(12 ; 21), are more reliably detected by FISH than by classical cytogenetics because the translocated portions 12p and 21q are virtually identical cytologically. When FISH was combined with Southern blotting and RT-PCR, the t(12;21) was identified as a recur-ring chromosomal abnormality in 16~25% of childhood B-lineage ALL. Despite the identification of the fusion partners in the t(12;21), the actual function of the TEL-AML1 and AML1-TEL fusion proteins in promoting malignant transformation is unclear. Whether the TEL-AML1 fusion alone is necessary and sufficient for malignant transformation in ALL and whether TEL inactivation has a role in leukemogenesis is currently unknown. Loss of the CDKN2(p16) gene at 9p21 is a common genetic abnormality in ALL and a variety of other malignancies. Homozygous CDKN2 deketions have been deteced in approximate 15% of B-lineage ALL and 75% of T-lineage ALL cases. The p16 protein functions as an inhibitor of cyclin-dependent kinase 4, and normally acts to stop cell cycle progression. Whether loss of p16 alone is necessary and sufficient for malignant transformation in childhood ALL has not been determined.
Arm ; Blotting, Southern ; Cell Cycle ; Child ; Chromosome Aberrations ; Chromosomes, Human, Pair 12 ; Clone Cells ; Cyclin-Dependent Kinase 4 ; Cytogenetics ; Genes, Tumor Suppressor ; Humans ; Loss of Heterozygosity ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Translocation, Genetic

PURPOSE: Of the cancers in childhood, leukemia is the most frequent one. For the desirable control of childhood leukemia, the basic data for the incidence has a great importance. The authors made a report about the incidence of leukemia in childhood, which analyzed the data from 126 cases in Kyongnam province, Korea, during 1991~1995. METHODS: The data were obtained from 126 new cases of childhood leukemia who had been living in the Kyongnam province and were diagnosed at the 26 university hospitals or general hospitals in the Kyongnam area and other cities from 1991 to 1995. RESULTS: The age-and-sex adjusted annual incidence rate per 100,000 population during 1991~1995 varied from 1.82 to 2.86, and cumulative annual incidence rate was 2.41 (male 2.26 and female 2.57 respectively). Male to female sex ratio was 1:1 in total cases. By the major types of childhood leukemia, the cases were composed of acute lymphocytic leukemia 70.6%, acute myelocytic leukemia 26.9% and chronic myelocytic leukemia 2.5%. The cumulative annual incidence rate per 100,000 population (crude rate) during 1991~1995 were 2.77 in Ulsan city, 2.62 in Chinju city and 2.34 in the whole area of Kyongnam province. CONCLUSION: It was concluded that the age-and-sex adjusted annual incidence rate per 100,000 of childhood in Kyongnam province was 2.41, which was lower than that in Pusan city in the same period. And, there was no significant difference of the cumulative annual incidence rate between Ulsan area and Chinju area in the same period.
Busan ; Female ; Gyeongsangnam-do* ; Hospitals, General ; Hospitals, University ; Humans ; Incidence* ; Korea* ; Leukemia* ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Leukemia, Myeloid, Acute ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Sex Ratio ; Ulsan

PURPOSE: Autografts with peripheral blood stem cells (PBSCT) have become an accepted alternative to bone marrow transplantation for restoring hematopoiesis after marrow ablative therapy. METHODS: Questionnaires are given to all the transplantation centers in Korea. Eleven centers reported 63 cases and retrospective analysis of these cases were done. RESULTS: Sixty-three children with acute myelogenous leukemia (AML) underwent PBSCT following cytoreductive chemotherapy at 11 transplant centers of the Korean Society of Pediatric Hematology-Oncology from January, 1996 through June, 2000. The patients consisted of 40 males and 23 females with a median age at PBSCT of 10 year (range 1~16). Peripheral blood stem cell (PBSC) were collected by a median of 5 apheresis per patient. Various kinds of multi-drug therapy as cytoreductive regimen were used, and the 'BCVAC' regimen consisting of BCNU, cyclophosphamide, VP-16 and cytarabine was used in 35 patients. The median number of infused mononuclear cell (MNC) and CD34 cells was 8.14 (0.3~26.6) 108/kg and 4.90 (0.12~46.9) 106/kg, respectively. Hematological recovery was evaluated in all patients. The median number of days required to achieve an absolute neutrophil count (ANC) of > 500/mm3, > 1,000/mm3 and platelet count of > 50 103/mm3 was 12 (8~48), 12 (9~84) and 35 (10~370), respectively. Sixteen patients relapsed 1 month to 18 months (median 2 months) after PBSCT, 1 patient progressed to secondary MDS 15 months after PBSCT and 1 patient died at d+39 due to CMV infection. So currently 45 patients are surviving disease free at 2 to 50 months (median 23 months). CONCLUSION: Even though the follow-up period was short and the number of patient was small the autologous PBSCT might be an alternative therapy in childhood AML.
Autografts ; Blood Component Removal ; Bone Marrow ; Bone Marrow Transplantation ; Carmustine ; Child ; Cyclophosphamide ; Cytarabine ; Drug Therapy ; Etoposide ; Female ; Follow-Up Studies ; Hematopoiesis ; Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells* ; Humans ; Korea* ; Leukemia, Myeloid, Acute* ; Male ; Neutrophils ; Platelet Count ; Surveys and Questionnaires ; Retrospective Studies ; Stem Cells

The Korean Society of Pediatric Hematology-Oncology surveyed pediatric allogeneic hematopoietic stem cell transplantation in Korea. From 1983 to April 2000, 267 children underwent allogeneic hematopoietic stem cell transplantation. Seventy-nine children were transplanted for acute myelogenous leukemia (AML), 76 for severe aplastic anemia (SAA), 62 for acute lymphoblastic leukemia (ALL), 44 for chronic myelogenous leukemia/myelodysplastic syndrome (CML/MDS) and 11 for nonmalignant rare disease. There were 152 males and 115 females with a median age of 9 years and median follow-up of 25 months. One hundred and eighty-nine of 267 cases were HLA-matched sibling transplants. The estimated event-free survival (EFS) of patients with SAA who underwent HLA-matched sibling transplants is 89%. The estimated EFS of ALL in CR1 and CR2 are 77% and 67%, respectively. The estimated EFS of AML in CR1 and CR2 are 73% and 60%, respectively. The estimated EFS of AML in CR1 prepared with Bu/Cy is 82%. The estimated EFS of CML/MDS is 71%. Eight out of 10 children with nonmalignant rare disease who underwent HLA-matched transplants are alive with disease free. Thirty-three children underwent unrelated bone marrow transplantation and 17 cord blood transplantation. Outcomes of patients with alternative stem cell sources are not estimated due to short median follow-up. These data shows that allogeneic hematopoietic stem cell transplantation is a curative method for children with hematopoietic stem cell disorders and we wish to share these results.
Anemia, Aplastic ; Bone Marrow Transplantation ; Child ; Disease-Free Survival ; Female ; Fetal Blood ; Follow-Up Studies ; Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells* ; Humans ; Korea ; Leukemia, Myeloid, Acute ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Rare Diseases ; Siblings ; Stem Cell Transplantation ; Stem Cells

Pichia ohmeri is an yeast-like fungus used in the food industry for fermentation. This organism has been implicated in human disease only in a few case reports. We describe herewith two cases of Pichia ohmeri fungemia in immunocompromised pediatric patients with central venous catheters. A 7-year-old patient with Burkitt's lymphoma undergoing chemotherapy and a newborn with low birth weight developed fungemia during hospitalizations. Both patients were receiving parenteral nutrition through central venous catheters. Both patients succumbed despite empiric treatment with amphotericin B in Case 1. A brief review of the literature ensues with the case reports.
Amphotericin B ; Burkitt Lymphoma ; Central Venous Catheters ; Child ; Drug Therapy ; Fermentation ; Food Industry ; Fungemia* ; Fungi ; Hospitalization ; Humans ; Infant, Low Birth Weight ; Infant, Newborn ; Parenteral Nutrition ; Pichia*

Self-healing reticulohistiocytosis is a perinatal disease characterized by rapidly developing and involuting benign histiocytic infiltration of the skin and other organ. We had recently experienced a case of a self-healing reticulohistiocytosis in 99-day-old infant who presented with multiple erythematous nodular lesions on face and trunk and pancytopenia. Our case differed from those previously reported in that extracutaneous involvement was found. The patient had pancytopenia, hypertriglyceridemia, hypofibrinogenemia hyperferritinemia and hepatosplenomegaly as well as skin lesions. Bone marrow showed a marked lymphocytosis with many histiocytes. We described a detailed clinical features of this case and reviewed the literatures.
Bone Marrow ; Histiocytes ; Humans ; Hypertriglyceridemia ; Infant ; Lymphocytosis ; Pancytopenia ; Skin

A 4-day-old patient with Down syndrome (DS) visited out patient department (OPD) because of jaundice and VSD. Peripheral blood smear showed 21% of myeloblast. After 4 weeks of observation, WBC count was 55,100/mm3 (blast 90%). BM aspirate showed AML (M7) and treatment was started with low dose Ara-C (20 mg/m2 for 21 days). After remission, maintenance therapy was done with low dose Ara-C (16 mg/m2 for 21 days), 6-TG (40 mg/m2 for 21 days) and low dose IL-2 (0.5 106U/m2 for 21 days) alternatively for 2 years. The patient remained in complete remission and VSD was corrected at 9 months of age. This case shows that remission can be achieved with low dose Ara-C and it can be maintained thereafter with low dose Ara-C, 6-TG and IL-2. Low dose IL-2 has the advantage of selectively activating immune cells with high affinity receptors, low treatment related morbidity, good compliance which can be injected at OPD. As the patients with DS have defect in IL-2 secretion, IL-2 may have an beneficial effects on treating AML in DS.
Compliance ; Cytarabine ; Down Syndrome* ; Drug Therapy* ; Granulocyte Precursor Cells ; Humans ; Interleukin-2* ; Jaundice ; Leukemia, Myeloid, Acute*

Acute lymphoblastic leukemia (ALL), in general, can be diagnosed by detecting blasts in peripheral blood or bone marrow. Some of the cases of ALL do not show typical leukemic features, and only manifest as refractory anemia, thrombocytopenia, myelofibrosis and lymphocytic infiltration into bone marrow. Several months after presentation, they may reveal typical leukemic features and are diagnosed as ALL. This kind of leukemia is called ALL with aleukemic prodrome. Although the incidence of ALL with aleukemic prodrome is 1.5~2.2% of childhood ALL cases, it is rarely reported in Korea. We experienced a 6 month-old female infant who presented with refactory anemia and thrombocytopenia, and two serial of bone marrow examination revealed only myelofibrosis. She subsequently developed ALL 3 months later. We report this case with a brief review of related literatures.
Anemia ; Anemia, Refractory ; Bone Marrow ; Bone Marrow Examination ; Female ; Humans ; Incidence ; Infant ; Korea ; Leukemia ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Primary Myelofibrosis ; Thrombocytopenia

PURPOSE: If primitive hematopoietic cells expanded ex vivo can be used in stem cell transplantation, duration for hematologic recovery will be shortened. In this study, CD34 cells were cultured with various hematopoietic growth factors which are known to stimulate proliferation of primitive hematopoietic cells. METHODS: CD34 cells isolated from cord blood were cultured and expanded ex vivo with IL-3, stem cell factor (SCF), flt-3 ligand (FL), thrombopoietin (TPO), granulocyte-colony stimulating factor (G-CSF). To find optimal combination of growth factors, CD34 cells were cultured for 9 days with various combinations of growth factors. To find optimal duration of culture, CD34 cells were cultured for 5, 7, 9, 12 days. To evaluate expansion of primitive hematopoietic cells, the number of CD34 cells, colony forming cells and long-term culture-initiating cells (LTC-IC) were counted by flow cytometry, colony forming cell assay and limiting dilution assay respectively. RESULTS: Primitive hematopoietic cells were successfully expanded from CD34 cells of cord blood. Maximal expansion of LTC-IC and CFU- GEMM were 2.58 and 2.37 fold respectively after 9 days of culture, and were obtained with the combination of IL-3 SCF FL TPO. When CD34 cells were cultured for 5, 7, 9, 12 days with the combination of IL-3 SCF FL TPO, expansion of LTC-IC was maximal (2.95 fold) after 9 days culture. After reaching maximal expansion of LTC-IC, the number of nucleated cells increased, but that of primitive hematopoietic cells decreased. CONCLUSION: Primitive hematopoietic cells can be successfully expanded ex vivo by using hematopoietic growth factors. Duration for hematologic recovery after stem cell transplantation will be shortened by using primitive hematopoietic cells expanded ex vivo.
Fetal Blood ; Flow Cytometry ; Intercellular Signaling Peptides and Proteins* ; Interleukin-3 ; Stem Cell Factor ; Stem Cell Transplantation ; Thrombopoietin ; Transplantation