PURPOSE: Chronic blood transfusions result in excessive iron deposition leading to eventual tissue damage and impaired function of organs, such as the liver, spleen, pancreas, skin, thyroid, and heart. We evaluated the body iron status and endocrinopathy in repeatedly transfused patients with aplastic anemia (AA). METHODS: Fourteen patients with AA who were transfused with more than 10 Units of packed RBC since 1996 were evaluated. We evaluated the correlation of amount of blood transfused with status of iron stores (determined by serum iron, TIBC, ferritin and transferrin saturation) and organ damage. RESULTS: Patients received a median of 61 units (range 11~168 units) of PRC. Twelve patients (85.7%) had elevated serum ferritin levels, and 11 (78.6%) had elevated transferrin saturation. Serum ferritin (P<0.01; r=0.868), and transferrin saturation (P<0.05; r=0.569) were significantly correlated with the amount of PRC transfused, respectively. Five patients had clinically significant iron overload despite the use of deferoxamine. Organ damage caused by transfusion iron overload were skin pigmentation (N=3), hepatic (N=1) and endocrinologic abnormalities. Diabetes (N=3), hypothyroidism (N=3), and hyogonadotropic hypogonadism (N=1) were observed. No patient developed clinically significant arthropathy or cardiac disease. CONCLUSION: AA patients who received chronic blood transfusions develop iron overload which may lead to endocrinopathy. Iron status and organ dysfunction should be monitored and effective measures to prevent iron overload should be applied in patients who need chronic transfusions.
Anemia, Aplastic* ; Blood Transfusion ; Deferoxamine ; Ferritins ; Heart ; Heart Diseases ; Hemochromatosis* ; Humans ; Hypogonadism ; Hypothyroidism ; Iron ; Iron Overload ; Liver ; Pancreas ; Skin ; Skin Pigmentation ; Spleen ; Thyroid Gland ; Transferrin

PURPOSE: Iron deficiency anemia (IDA) is the most common nutrient deficient disorder in infants and young children. Iron deficiency at this age group can cause serious effects on mental and psychomotor development. We analyzed the hematologic profiles of infants and young children with IDA, comparing them with control group. METHODS: The feeding practices and the iron batteries investigated in 198 anemic patients aged 5 to 36 months who had been brought to Inha General & University Hospital. Control group were 129 healthy infants and children who visited DongBu Municipal Hospital and the local health center for immunizations. They also had hemoglobin concentration (Hb), hematocrit, mean corpuscular volume (MCV), mean corpuscular hemoglobin and red cell distribution width (RDW) tested by the electron counters. Patients with hemoglobin level <11 g/dL who had serum ferritin <10 ng/mL or transferrin saturation <15% were classified as having IDA. RESULTS: Out of the 198 subjects (M:F=1.6:1) with IDA, 81.8% (n=162) was breast feeding more than 6 months. The main causes which they were brought to the clinic were infectious or inflammatory illness, and only 13.1% with IDA were visited for evaluation of pallor or anemia. Correlation between Hb and MCV was much more in IDA group than control group (r=0.709, r=0.368; P<0.001). CONCLUSION: By combining Hb with MCV and RDW as well as iron batteries in screening for iron deficiency and IDA, the accuracy of diagnosis can be increased. We support the use of appropriately iron-fortified formulas or weaning foods, or the routine iron supplement starting at 6 months of age in exclusively breast-fed infants to prevent the iron deficiency.
Anemia ; Anemia, Iron-Deficiency* ; Breast Feeding ; Child* ; Diagnosis ; Erythrocyte Indices ; Erythrocytes* ; Ferritins ; Hematocrit ; Hospitals, Municipal ; Humans ; Immunization ; Infant* ; Iron* ; Mass Screening ; Pallor ; Transferrin ; Weaning

PURPOSE: The c-myb protooncogene encodes MYB protein that is critical for normal and leukemic hematopoietic cell proliferation and development. It is known that c-myb plays an important role in leukemogenesis as well. Aberrant expression of c-myb is seen in acute myeloid leukemia (AML), acute lymphoid leukemia (ALL), and chronic myeloid leukemia (CML). We reasoned that down regulation of c-myb expression using synthetic antisense oligomers targeted to c-myb mRNA might prove useful antileukemic agents if leukemia cells were more dependent on c-myb function for proliferation than their normal counterparts. To investigate the applying possibility of c-myb antisense oligodeoxynucleotides as the useful antileukemic agents, we examined the cell viability, cloning efficiency and the expression of c-myb mRNA and MYB protein after the exposure of c-myb oligomers on normal bone marrow cells and leukemia cells. MATERIALS AND METHODS: We maintained in short-term suspension culture for 4 days to analyze the effect of c-myb oligomers on normal bone marrow cells and chronic myelocytic leukemia cell line K562. During suspension culture, cell counts and viability were periodically determined, and immediately seeded into duplicate methylcellulose cultures containing recombinanat human interleukin 3 and GM-CSF. Cells placed in semisolid cultures were allowed to grow for an additional 10~12 days. We counted the colonies, and then RNA and protein was extracted from cells cloned in liquified methyl- cellulose cultures. We detected the c-myb mRNA expression by RT-PCR and Southern hybridization analysis and MYB expression by Western hybridization analysis. RESULTS: c-myb sense oligomers had negligible effects on K562 cells growth in short- term suspension culture, while exposure to c-myb antisense oligomers resulted in a daily decline in cell number. In contrast, normal bone marow cell viability and numbers were unaffected by c-myb sense or antisense oligomers exposure. c-myb antisense oligodeoxy nucleotides strongly inhibited or completely abolished growth and colony formation of K562 cells. In contrast, c-myb sense oligomers did not affect. At antisense dose that inhibited leukemic cell growth, normal bone marrow cells survived. Thus, normal and leukemic cells showed the differential sensitivity to the toxic effect of c-myb antisense oligomers. RT-PCR, Southern hybridization analysis and Western hybridization analysis of c-myb antisense-treated K562 cells revealed a complete absence of c-myb mRNA expression and MYB expression. CONCLUSION: Results obtained from these studies suggest that inhibition of c-myb function with antisense oligodeoxynucleotides might eventually form the basis for a molecular biologic approach to leukemia therapy, perhaps most immediately as ex vivo bone marrow purging agent.
Bone Marrow Cells ; Bone Marrow Purging ; Cell Count ; Cell Line ; Cell Proliferation ; Cell Survival ; Cellulose ; Clone Cells ; Cloning, Organism ; Down-Regulation ; Granulocyte-Macrophage Colony-Stimulating Factor ; Humans ; Interleukin-3 ; K562 Cells* ; Leukemia ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Leukemia, Myeloid, Acute ; Methylcellulose ; Nucleotides ; Oligodeoxyribonucleotides* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; RNA ; RNA, Messenger

PURPOSE: The widely used chemotherapeutic agents exert their anti-cancer effects by the inducing of apoptosis in sensitive tumor cells. Recently, the protective effect of zinc ion on apoptosis has been reproted. However, it is not well understood about the effects of zinc ion on the anticancer drug-induced apoptosis. In general, zinc inhibits a nuclear endonuclease, thereby causing inhibition of apoptosis. In addition, there is other possibility that zinc can prevent apoptosis at earlier stage such as the activation of caspase-3 than that of the activation of endonuclease. Therefore, we investigated the effects of zinc ion on the apoptosis of HL-60 cells caused by adriamycin (ADR). METHODS: HL-60 cells were cultured in RPMI 1640 and treated with various concentrations and time periods of ADR with or without pretreatment of zinc ion. Cellular DNA was extracted and analyzed by electrophoresis on a 1.5% agarose gel to detect DNA fragmentation. The activity of caspase-3 was measured by the proteolytic cleavage of the fluorogenic substrate DEVD-AMC. Poly-ADP-ribose polymerase (PARP) cleavage was analyzed by western blotting using anti-PARP antibody. RESULTS: ADR induced the apoptotic death of HL-60 cells in a dose and time dependent manner, which was characterized by increasing ladder-pattern DNA fragmentation. Pretreatment of HL-60 cells with zinc ion caused potent inhibition of ADR-induced apoptosis. Consistent with apoptotic death of HL-60 cells, ADR induced the catalytic activation of caspase-3. After pretreatment of zinc ion, the activation of caspase-3 and the proteolysis of PARP induced by ADR were markedly inhibited. CONCLUSION: These results indicate that zinc ion prevents the ADR-induced apoptosis of HL-60 cells through an inhibition of caspase-3 activity, which occurs upstream from the activation of endonuclease.
Apoptosis* ; Blotting, Western ; Caspase 3 ; DNA ; DNA Fragmentation ; Doxorubicin ; Electrophoresis ; Fluorescent Dyes ; HL-60 Cells* ; Humans ; Proteolysis ; Sepharose ; Zinc*

PURPOSE: This study was carried to examine the temporal trend and geographical distribution of the childhood cancers in Taegu and Kyungpook province and to postulate an etiological hypothesis for development of the childhood cancer. METHODS: A total of 799 childhood cancer patients whose addresses were either Taegu or Kyungpook province were diagnosed at 5 major hospitals in Taegu from January 1982 to December 1996. The types, sexes, years, and frequencies of the childhood cancer and regional distributions were analyzed, based on the hospital records of these patients. RESULTS: The most common childhood cancer was leukemia that accounted for 49.2% of all childhood cancer cases and it was followed by CNS tumor (12.3%), lymphoma (8.4%), neuroblastoma (7.4%), Wilms tumor (3.9%), retinoblastoma (3.4%), rhabdomyo sarcoma (2.7%), bone tumor (2.4%), embryonal carcinoma (1.9%), hepatoblastoma (1.3%) and others (7.1%). Male to female ratio of the cases was 1.5:1. The changes of the annual incidence rates over 15 years in Taegu and Kyungpook area were not consistently increasing but rather variable. Cancer incidence rate of Taegu was significantly higher than that of Kyungpook province (P<0.005). The incidence rates of industrialized cities around Taegu were significantly higher than those of agricultural regions of northern Kyungpook (P<0.05). CONCLUSION: Geographical difference in cancer incidence rate suggested that certain environmental factors may be associated with the childhood cancer. To identify such factors an analytical epidemiologic study is warranted. For the analytical epidemiologic study, a detailed history of residential area and occupational history of parents should be recorded uniformly for all the new childhood cancer cases.
Carcinoma, Embryonal ; Daegu* ; Epidemiologic Studies ; Epidemiology ; Female ; Gyeongsangbuk-do* ; Hepatoblastoma ; Hospital Records ; Humans ; Incidence ; Leukemia ; Lymphoma ; Male ; Neuroblastoma ; Parents ; Retinoblastoma ; Sarcoma ; Wilms Tumor

PURPOSE: This study was undertaken to investigate the clinical characteristics and prognostic predictors of myelodysplastic syndrome (MDS) in childhood. Method: The characteristics and laboratory findings of 20 patients seen at Asan Medical Center for the past 10 years from September 1989 to August 1998 were reviewed retrospectively with regard to the new International Prognostic Scoring System (IPSS) proposed by International MDS Risk Analysis Workshop. RESULTS: 1) In 20 children with MDS we studied, there was no age or sex predilection unique to the subgroups of MDS. 2) 19 cases (95%) out of the 20 had pallor at the time of diagnosis. Other major clinical findings were bleeding tendency in 11 (55%), fever in 8 (40%), hepatosplenomegaly in 8 (40%), and lymphadenopathy in 3 (15%). 3) The hemoglobin level was less than 10 g/dL in all cases and absolute neutrophil count (ANC) was decreased in 11 cases, thrombocytopenia in 15 cases. Pancytopenia was noted in 8 cases (40%). 4) Of the 20 cases, 9 had refractory anemia (RA), 3 refractory anemia with excess blasts (RAEB), 3 refractory anemia with excess blasts in transformation (RAEBIT), and 5 juvenile chronic myelogenous leukemia (JCML). 5) All RA patients were Intermediate (INT)-1 risk group, and all RAEB children were INT-2 risk group. The 3 cases of RAEBIT fell into INT-1, INT-2, and high risk group. Three cases of JCML were INT-1 group, and 2 cases INT-2 group. 6) Seven cases out of 13 INT-1 group had mean survival of 20.2 month (6~57 month), but only 1 out of 6 INT-2 survived. One case of high risk group succumbed to disease 50 months after diagnosis. CONCLUSION: These results showed that there was no age or sex predilection for the specific subgroup of childhood MDS. All the FAB subtypes of the childhood MDS except RA subgroup had poor survival. In this study, we found the IPSS seemed to be a prognostic predictor in childhood MDS but more cases are needed to confirm the validity of IPSS.
Anemia, Refractory ; Anemia, Refractory, with Excess of Blasts ; Child ; Chungcheongnam-do ; Diagnosis ; Education ; Fever ; Hemorrhage ; Humans ; Leukemia, Myelomonocytic, Juvenile ; Lymphatic Diseases ; Myelodysplastic Syndromes* ; Neutrophils ; Pallor ; Pancytopenia ; Prognosis ; Retrospective Studies ; Thrombocytopenia

PURPOSE: This study was aimed at assessing the difference of the prevalence of iron deficiency and iron deficiency anemia among rural and urban middle school students in relation to dietary habit. METHODS: With a questionnaire, blood samples were obtained from 439 apparently healthy rural and urban middle school students residing in Ulsan. Anemia was defined as hemoglobin level of 12.6 g/dL or less for boys and 11.9 g/dL or less for girls. Iron deficiency was defined as serun ferritin level less than 12 micrograms/L or/and transferrin saturation less than 14%. Iron deficiency anemia was defined as iron deficiency plus low hemoglobin. RESULTS: 1) In boys, the prevalence rate of anemia was 17.2%. Among these anemias, 5.4% were found to be iron deficiency anemia. In girls, the prevalence of anemia increased with age. The prevalence of iron deficiency anemia was 6.9%. 2) In girls, the prevalence rate of anemia in rural area was higher than that of anemia in urban area (12.6% in rural, 6.1% in urban, P<0.01). 3) The prevalence of anemia and iron deficiency in the students with menstruation was 10.6% and 33.1%, which was higher than the prevalence of 2.5% and 7.5% in those who did not have the menarche (P<0.001 and P<0.001, respectively). 4) Dietary intake of rural and urban middle school students was estimated lower in energy, iron than the recommeded dietary allowance (RDA). In girls, dietary intake of rural middle school students was estimated lower in iron, niacin, and vitamin C than that of urban middle school students. 5) Nutritional factors such as energy, carbohydrate, protein, and phosphorus showed positive correlation with RBC, hemoglobin (P<0.05). CONCLUSION: It is recommended to enforce the nutritional education to take enough iron in middle school students to reduce the high prevalence rate of anemia among pubertal students.
Anemia* ; Anemia, Iron-Deficiency ; Ascorbic Acid ; Education ; Female ; Ferritins ; Food Habits ; Humans ; Iron* ; Menarche ; Menstruation ; Niacin ; Phosphorus ; Prevalence ; Surveys and Questionnaires ; Transferrin ; Ulsan*

No available abstract.
Leukemia, Myeloid, Acute*

No available abstract.
Leukemia, Promyelocytic, Acute*

No available abstract.
Japan* ; Leukemia, Myeloid, Acute*