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Phenotypic Analysis of Lymphocyte Subpopulation in Cord Blood.

Chan Wook WOO ; Kwang Chul LEE ; Yoon Jeong CHO ; Young Tae KIM

Korean Journal of Pediatric Hematology-Oncology.1997;4(1):118-125.

BACKGROUND: Cord blood transplantation has generated much enthusiasm because of the low incidence of GVHD(graft-versus-host disease), even in HLA(Human Leukocyte Antigen) mismatched situations, owing to intrinsic defects in mature T cell functions, and human cord blood is a constantly and easily obtainable source for hematopoietic stem cell. This study was undertaken to elucidate the possible mechanisms of low GVHD incidence observed when cord blood is used for stem cell transplantation. Based on current knowledge of the phenotypic correlates with functional subtypes of peripheral blood lymphocytes, we evaluated lymphocyte subpopulation in cord blood. METHODS: The 25 cord blood and 20 peripheral blood of adult control were included in this study. Monoclonal antibodies(MAb) conjugated with fluorescein-isothiocynate(FITC), phycoerythrin(PE) were used in this study. Heparinized whole blood was stained with mAbs using the whole blood lysis method. We used FACScan flow cytometer with argon laser tuned at 488 nm. Analysis was done by either Simulset or lysis II research software. RESULT: 1) The means of WBC and lymphocyte content were higher in cord blood than in adult blood(p<0.05). 2) The overall percentages of cord blood T(CD3+) cells, B(CD19+) cells, NK(natural killer)(CD16+/CD56+) cells were not different from adult controls. 3) The CD4+ : CD8+ ratio of cord blood was higher than thar of adult controls(2.7 in cord bloods versus 1.8 in adults). 4) The majority of the cord blood cells, both CD4+ and CD8+, co-expressed the CD45RA+ (naive) molecule. 5) The CD57, an antigen present on a subpopulation of NK cells, is absent in cord blood. CONCLUSION: We determined that cord blood contained mainly naive T lymphocytes to be predominantly suppressor cells. We found reduced alloproliferative, allostimulatory and allocytotoxic capacity of cord blood lymphocytes. Therefore, the GVHD may be less readily induced in cord blood transplantation, even in the face of HLA difference.
Adult ; Argon ; Fetal Blood* ; Hematopoietic Stem Cells ; Heparin ; Humans ; Incidence ; Killer Cells, Natural ; Leukocytes ; Lymphocyte Subsets* ; Lymphocytes* ; Phenotype ; Stem Cell Transplantation ; T-Lymphocytes

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Effect of Preoperative Chemotherapy.

Jae Kyung LEE ; Hyeong Soo CHOI ; Ki Woong SUNG ; Hee Young SHIN ; Hyo Seop AHN

Korean Journal of Pediatric Hematology-Oncology.1997;4(1):105-117.

BACKGROUND: The survival rate of children with neuroblastoma has been improved over 20 years, excluding the metastatic disease, in which it does not exceed 20% so far. New treatment modalities have been developed to improve the outcome in metastatic disease. Preoperative chemotherapy reduce the size, the vascularity and the adhesiveness, so increase the resectability of the primary tumor. This retrospective clinical study was designed to review the survival rate in neuroblastoma and to analyze the effect of preoperative chemotherapy in the view point of neoadjuvant therapy on long-term survival in advanced disease. METHODS: One hundred and thirty-four cases were reviewed from 135 patients with neuroblastoma registered at the Department of Pediatrics in Seoul National University Children's Hospital from January, 1985 till December, 1995. The survival rate was reviewed according to the stage. The age, sex of the patients, the stage, anatomical site of the tumor, the level of serum ferritin and neuron-specific enolase were analyzed for the risk factors on survival. RESULTS: Ranges of age at diagnosis were from 1 month to 166 months with the median of 39 months. Five year survival rates and five year disease-free survival rates were 100%, 100% in stage 1(n=5), 90.9%, 90.9% in stage 2(n=13), 43.4%, 40.6% in stage 3(n=19), 27.1%, 19.8% in stage 4(n=95) and 100%, 100% in stage 4S(n=2), respectively. In stage 3, five year survival rate was 52.5% in group receiving neoadjuvant chemotherapy, 28.6% in control group(P=0.02). Five year disease-free survival rate was also noted as 48.6%, 28.6% in each group(P=0.02). In stage 4, five year and ten year survival rates were 27.6%, 23.6% in group receiving neoadjuvant chemotherapy, 26.9%, 0% in control group(P=0.02). Five year and ten year disease-free survival rates were 14.3%, 14.3% in group receiving neoadjuvant chemotherapy, 20%, 0% in control group (P=0.11). In univariate analysis, the age, the stage, and the site of primary tumor appeared to affect the long-term survival. CONCLUSION: Neoadjuvant chemotherapy and delayed primary surgery contribute for advance in survival in advanced neuroblastoma via increasing the resectability of the primary tumor.
Adhesiveness ; Child ; Diagnosis ; Disease-Free Survival ; Drug Therapy* ; Ferritins ; Humans ; Neoadjuvant Therapy ; Neuroblastoma ; Pediatrics ; Phosphopyruvate Hydratase ; Retrospective Studies ; Risk Factors ; Seoul ; Survival Rate

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Effect of Ganciclovir on Growth of Neuroblastoma Cell Line Expressed HSV-TK Gene.

Hyun Sang CHO ; Chuhl Joo LYU ; Chang Hyun YANG ; Seung Hwan OH ; Kun Hong KIM ; Kir Young KIM ; Byung Soo KIM

Korean Journal of Pediatric Hematology-Oncology.1997;4(1):98-104.

BACKGROUND: Replication of defective adenoviral vectors can be used for gene transfer into a wide spectrum of replicating and nonreplicating cells. Accordingly, selective introduction of genes encoding for susceptibility to nontoxic drugs into proliferating tumor may be used to treat cancer. We investigated the efficacy of in vitro transduction of neuroblastoma cell with the herpes simplex virus thymidine kinase gene followed by administration of the antiviral drug ganciclovir. METHODS: The recombinant adenoviral vector ADV/TK carrying the HSV-TK gene, under the control of the promoter from Rous sarcoma virus long term terminal repeat was constructed. And 1 x 10(4) Neuro 2a cells were plated in 96 well cultured plates and infected with ADV/TK at multiplicity of infection of 0, 1, 10, and 100. Twenty-four hours later, the infected cells were treated with PBS or ganciclovir at a concentration of 10 g/ml. After 48hr, the surviving cells in 96 well plates were determined by MTT assay. RESULTS: After infection in vitro with ADV/TK at moi of 0, 1, 10, 100 and subsequent ganciclovir treatment, the percent survival rate of 1 x 10(4) Neuro 2a cells were 105%, 32%, 25%, and 15%. But the survival rate of 1 x 10(4) Neuro 2a cells with PBS treatment were 100%, 92%, 105%, 103%. CONCLUSION: We concluded that in vitro transduction of neuroblastoma cell with the herpes simplex-thymidine kinase gene followed by administration of the antiviral drug ganciclovir is very effective.
Cell Line* ; Ganciclovir* ; Neuroblastoma* ; Phosphotransferases ; Rous sarcoma virus ; Simplexvirus ; Survival Rate ; Terminal Repeat Sequences ; Thymidine Kinase

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Ifosfamide and Etoposide in Relapsed Refractory Childhood Acute Lymphoblastic Leukemia.

Shung Shin KIM ; Bin CHO ; Dae Chul JEONG ; Hack Ki KIM

Korean Journal of Pediatric Hematology-Oncology.1997;4(1):90-97.

BACKGROUND: The prognosis for children with relapsed acute lymphoblastic leukemia remains dismal. Ifosfamide has previously been shown to be active as a single agent and in combination with doxorubicin, etoposide, and teniposide in pediatric solid tumors, recurrent acute lymphoblastic leukemia and adult acute leukemia. We assessed the efficacy and the toxicity of the drug combination with ifosfamide and etoposide in patients with relapsed refractory acute lymphoblastic leukemia. METHODS: Between April 1995 and May 1996, twenty children aged 1 to 14 years with ALL in Catholic Medical Center, all heavily pretreated and in bone marrow relapse, were enrolled in this study. Drugs were given intravenously each day for 5 days at the following doses ; ifosfamide 1.8 g/m2/day, etoposide 100 mg/m2/day and mesna 1440 mg/ m2/day(as a uroprotectant) ; Cycles were repeated every 28 days for two cycles. RESULTS: 1) Twenty heavily pretreated patients were entered on study. At study entry, seventeen patients were in first relapse, two were in second relapse and one was in third relapse. 2) Six patients(30%) achieved complete remission, and eight patients(40%) achieved partial remission. Overall response rate was 70%. 3) Duration of remission ranged from 30 days to 230 days. 4) The toxicity of the regimen was tolerated. Moderate or severe toxicity evaluated on a per cycle basis included : neutropenia 52.5%, thrombocytopenia 45%, hemorrhagic cystitis 12.5% and mucositis 2.5%. 5) Two patients went on to bone marrow transplantation with histocompatibility matched sibling donors while in remission. CONCLUSION: The combination of ifosfamide and etoposide with mesna uroprotection has significant activity in relapsed refractory childhood lymphoblastic leukemia with tolerable toxicity. We recommended bone marrow transplantation after successful reinduction because of short remission duration of this regimen.
Adult ; Bone Marrow ; Bone Marrow Transplantation ; Child ; Cystitis ; Doxorubicin ; Etoposide* ; Histocompatibility ; Humans ; Ifosfamide* ; Leukemia ; Mesna ; Mucositis ; Neutropenia ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Prognosis ; Recurrence ; Siblings ; Teniposide ; Thrombocytopenia ; Tissue Donors

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A Clinical Observation of Childhood Myelodysplastic Syndrome.

Sun Young PARK ; Pil Geung WHANG ; Soon Yong LEE

Korean Journal of Pediatric Hematology-Oncology.1997;4(1):78-89.

BACKGROUND: Childhood myelodysplastic syndrome(MDS) is a heterogenous disease complex which has characteristics of cytopenia in one or more hemopoietic cell lines in peripheral blood and of dysmorphisms of hemopoietic precursors in bone marrow, but quite different from adult one. We experienced 22 patients with childhood myelodysplastic syndrome during 9 years from 1987 to 1995 in the Department of Pediatrics, Inje University College of Medicine, Pusan Paik Hospital, Pusan, Korea and performed a clinical study about them to analyze the clinical and hematological features and treatment outcome retrospectively. METHOD: We analyzed their clinical features including age and sex distribution, chief complaints and physical findings on first admission, distribution of subtypes, hematologic features including initial hemoglobin, total WBC and platelet count, and treatment outcome including leukmic transformation and prognostic scores. RESULTS: 1) Of the total 22 patients, 10 were in 0-4 years of age on first admission, 8 in 5-9, and 4 above 10 years of age. The male : female sex ratio was 2.1 : 1. 2) Of the 22 patients, chief complaints on first admission were bleeding tendency in 9 patients(40.9%), pallor in 8(36.4%) and fever in 3(13.6%), in order, and physical findings on first admission were purpura or ecchymoses in 17(77.3%), anemia in 15(68.2%) and hepatosplenomegaly in 8(36.4%), in order. 3) Hematologic findings on first admission were as follows : hemoglobin levels were below 3 g/dl in 1 patient(4.5%), 3-6 g/dl in 10(45.5%), and 6-9 g/dl in 11(50.0%). Initial WBC counts were below 5,000/mm3 in 13 patients(59.1%), 5,000-10,000/mm3 in 5(22.7%) and above 10,000/mm3 in 4(18.2%). Initial platelet counts were below 20,000/mm3 in 10(45.5%), 20,000-50,000/mm3 in 5(22.7%), 50,000-100,000/mm3 in 5(22.7%) and above 100,000/mm3 in 2(9.1%). 4) Of the 22 patients, 12 patients(54.6%) were RA type, 1(4.6%) RAS, and 3(13.6%) RAEB, RAEB-T and JCML types, respectively. 5) According to prognostic scores by Mufti et al(1986), none were in 'good' group, 17 patients(89.5%) in 'intermediate' group with 39.5 months of mean duration of survival(range 4-95 months) and 2(10.5%) in 'poor' group with 18 months of mean duration of surviral(range 17-19) until the last follow-up. However, the subtypes and clinical status seemed not to be related to the prognostic scores. 6) Sixteen patients were treated with low dose cytosine arabinoside(10 mg/m2/12hrs), of whom 7 patients gained long-standing event-free survival, whose treatment regimen was changed to oral 6-TG about 2 years later. All 3 of JCML were treated with A-Triple-V regimen, one of whom was died of sepsis, one was transformed into AML and died of sepsis, while the remained one gained long-standig event-free survival (62 months). 7) Leukemic transformation into AML occured in 7 patients(RA 1, RAEB 2, RAEB-T 3, JCML 1), 6 of whom were dead, while one gained long-standing event-free survival of 34 months. CONCLUSION: We concluded that RA was the most dominant type among our patients, and the frequency to transform into AML was 31,8%, and 31.9% of the patients had long-term survival, and that reliability of prognostic scoring system by Mufti et al(1986) was not high.
Adult ; Anemia ; Anemia, Refractory, with Excess of Blasts ; Bone Marrow ; Busan ; Cell Line ; Cytosine ; Disease-Free Survival ; Ecchymosis ; Female ; Fever ; Follow-Up Studies ; Hemorrhage ; Humans ; Korea ; Male ; Myelodysplastic Syndromes* ; Pallor ; Pediatrics ; Platelet Count ; Purpura ; Retrospective Studies ; Sepsis ; Sex Distribution ; Sex Ratio ; Treatment Outcome

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A Clinical Study of Fanconi's Anemia.

Sung Ho CHO ; Hoon KOOK ; Geun Mo KIM ; Won Sang YOON ; Tae Hyung CHO ; Tai Ju HWANG

Korean Journal of Pediatric Hematology-Oncology.1997;4(1):70-77.

BACKGROUND: Fanconi's anemia(FA) is an autosomal recessive disease characterized by aplastic anemia and congenital malformations. As up to 30% of patients have no physical stigmata, the modern diagnosis of FA rests on chromosomal breakage of patient's cells induced by chemical clastogens such as diepoxybutane(DEB) or mitomycin-C(MMC). METHODS: We reviewed the clinical manifestations, laboratory findings, diagnostic methods, treatment and outcome of 6 patients diagnosed to have a FA at the Chonnam University Hospital for the last 6 years. RESULTS: Six cases(16.2 %) were found to have FA among 37 aplastic children who were diagnosed during the same period. The mean age at diagnosis was 6.3 years which was the usual onset of hematologic findings. All patients had features of aplastic anemia, and had one or more anomalies, such as low birth weight, hyperpigmentation, cafeau-lait spots, mental retardation, developmental delay, peculiar face(broad nasal bases, epicanthal folds, micrognathia), polydactyly, microcephaly, short stature, and dislocation of hip. We found increased breaks in cultured cells with DEB and MMC in 5 cases tested. The median duration of follow-up was 30 months. Oxymetholone and prednisolone treatment was partially beneficial in three cases. Immunosuppressive treatment with ALG/ATG was not successful in two cases tried. Four cases are living now, without transfusion in three. Two patients were died of disseminated fungal infection and transplant-related problems, respectively. CONCLUSIONS: Fanconi's anemia should be sought carefully in any patients with aplastic anemia because the prognosis, treatment modality, and the approach to bone marrow transplantation are quite different when the hematologic disorder is inherited rather than acquired.
Anemia, Aplastic ; Bone Marrow Transplantation ; Cells, Cultured ; Child ; Christianity ; Chromosome Breakage ; Diagnosis ; Dislocations ; Fanconi Anemia* ; Follow-Up Studies ; Hip ; Humans ; Hyperpigmentation ; Infant, Low Birth Weight ; Infant, Newborn ; Intellectual Disability ; Jeollanam-do ; Microcephaly ; Mitomycin ; Mutagens ; Oxymetholone ; Polydactyly ; Prednisolone ; Prognosis

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Effect of Desferrioxamine Therapy in Patients with Transfusional Hemosiderosis Due to Severe Aplastic Anemia.

Jung Hyun LEE ; Bin CHO ; Dae Chul JEONG ; Hack Ki KIM

Korean Journal of Pediatric Hematology-Oncology.1997;4(1):62-69.

BACKGROUND: This study was carried out to evaluate the efficacy of desferrioxamine as a chelating agent in iron overloaded patients with severe aplastic anemia due to multiple transfusion. METHODS AND MATERIALS: From Oct. 1995 to Aug. 1996, 15 patients with aplastic anemia, diagnosed from May 1995 to Jan. 1996 at St. Mary's Hospital, who had a transfusional hemosiderosis were included in this study. They received 19 courses of high-dose desfer-rioxamine therapy for 6 days(20 to 30 mg/kg daily as a 24-hour intravenous infusion) . Before and after treatment, we measured serum ferritin, iron, TIBC, 24-hour urinary excretion of iron. RESULTS: 1) The range of iron load before treatment was between 4.5 and 20.0 gram. 2) Because of limit of detection(1,800 microgram/L), it was difficult to compare the changes of serum ferritin level after therapy to those of before therapy. 3) There was no significant differences between the levels of serum iron before and after therapy(214.3+/-62.8 vs 220.0+/-53.3). And there was no significant differences between TIBC before and after therapy(235.8+/-64.6 vs 259.4+/-60.1). 4) Iron/TIBC ratios were significantly deceased after desferrioxamine treatment compared to those of before therapy(0.90+/-0.04 vs 0.85+/-0.04, P<0.001) and mean urinary excretions of iron were increased by high-dose desferrioxamine compared to those by test dose(6.5+/-7.6 vs 29.1+/-14.3, P<0.001) CONCLUSION: High-dose desferrioxamine therapy is very effective for chelating and excretion of iron in iron overloaded patients with severe aplastic anemia due to multiple transfusion. A repeat administration of desferrioxamine is necessary for the iron overloaded patient to eliminate the risk of a transfusional hemosidersis.
Anemia, Aplastic* ; Deferoxamine* ; Ferritins ; Hemosiderosis* ; Humans ; Iron ; Iron Overload

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Predictors of Chronicity in Childhood Idiopathic Thrombocytopenic Purpura.

Young Tak LIM ; Seung Hun KIM ; Jae Hong PARK ; Su Young KIM

Korean Journal of Pediatric Hematology-Oncology.1997;4(1):55-61.

BACKGROUND: Acute idiopathic thrombocytopenic purpura(ITP) in children has a low morbidity and mortality, and most of cases are self limited illness. But some of them are not responsive to treatment and finally progress to chronic disease. The purpose of this study is to evaluate the factors which influence the outcome in childhood idiopathic thrombocytopenic purpura. METHOD: From January 1990 to December 1995, patients with ITP who were admitted to the Pediatric department of Pusan National University Hospital were divided into acute and chronic form depending on whether the platelet count had returned to normal(150 x 10(9)/1) by six months after diagnosis. Between two groups, the clinical and laboratory characteristics, response to treatment were compared. RESULT: 1) Out of 55 patients with ITP, 36 cases(65.5%) were acute and 19 cases(34.5%) were chronic ITP. 2) Sex, previous viral infection history, clinical manifestations were not significantly different between two groups, but the chronic ITP was rare in less than 1 year of age. 3) The duration of symptoms over >2 weeks at presentation was strongly predictive of chronic ITP. 4) Most of chronic disease (87.5%) showed platelet counts below 150 x 10(9)/1 at 28 days after diagnosis as compared with acute disease(25.0%). 5) Among 19 cases of chronic disease, 6 cases responded completely to treatment, but 11 cases(57.9%) did not respond to any treatments, 2 cases improved spontaneously. CONCLUSION: A history of symptoms over 2 weeks at presentation and platelet counts below 150 x 10(9)/1 at 28 days after diagnosis were strong predictors of chronic ITP in children.
Busan ; Child ; Chronic Disease ; Diagnosis ; Humans ; Mortality ; Platelet Count ; Purpura, Thrombocytopenic, Idiopathic*

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A Role of gamma-subunit in FcgammaRII Signal Transduction of Platelets.

Hwang Min KIM ; Baek Keun LIM

Korean Journal of Pediatric Hematology-Oncology.1997;4(1):48-54.

BACKGROUND: The FcgammaRII receptor, the only kind of Fc receptor in the platelet, plays important role for immune-complex mediated platelet activation, which occurs in Heparin induced thrombocytopenia(HIT) or antiphospholipid syndrome. The purpose of this research is to find out the role of gamma-subunit in FcgammaRII signal transduction of platelets. METHODS: After cross-linking the FcgammaRII receptors with IV.3 anti FcgammaRII specific antibody and Rabbit anti mouse antibody, we made the platelet lysate in the RIPA EDTA lysis buffer condition, and performed anti gamma-subunit immunoprecipitation. After polyacylamide gel electrophoresis and Western transfer, we probed the nitrocellulose membrane of gamma-subunit immunoprecipitate with anti gamma-subunit antibody. RESULTS: The mobility shift of gamma-subunit was observed upon FcgammaRII cross-linking. CONCLUSION: The gamma-subunit is activated upon FcgammaRII cross-linking, which means the gamma-subunit is involved in FcgammaRII signal transduction of platelet.
Animals ; Antiphospholipid Syndrome ; Blood Platelets ; Collodion ; Edetic Acid ; Electrophoresis ; Heparin ; Immunoprecipitation ; Membranes ; Mice ; Platelet Activation ; Receptors, Fc ; Signal Transduction*

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Management for Neuroblastoma Infants in Japan.

Takafumi MATSUMURA ; Tadashi SAWADA ; Takuma SHIKATA ; Yoshifumi MATSUMOTO ; Tomoko IEHARA

Korean Journal of Pediatric Hematology-Oncology.1997;4(1):18-28.

INTRODUCTION: The introduction of the mass screening (MS) program measuring urinary catecholamine metabolites at six-months of age for detecting neuroblastoma has resulted in the increase in both number and incidence of patients detected less than one year of age in Japan. The prognosis for infantile neuroblastoma is well known to surpass that for older patients. The prognosis of patients detected by MS has been outstanding. However, in Japan, there has been no consentient guideline of optimal therapeutic management for infants with favorable prognosis. There has been a continuing controversy on the selection of appropriate therapy for neuroblastoma infants, especially those detected by MS. In Japan, based on prognostic factors including N-myc amplification as well as clinical stage, patients with advanced disease receive a consistent therapeutic regimen. In contrast, neuroblastoma infants with favorable biological characteristics and clinical outcome have received variable therapeutic regimens at individual institutions. Resulting from an urgency to assess the status and enforce a consentient as well as an optimal management plan for neuroblastoma infants, the survey and the analysis on a total of 537 cases, including 355 cases detected by the MS program, were conducted and led us to the conclusion that neuroblastoma infants in Japan had been treated comparatively intensive despite excellent prognosis, and further that adjuvant chemotherapy should be avoidable or minimized for patients with such excellent outcomes. Finally, a nationwide prospective study (#9405) has been commenced in Japan to standardize and to optimize therapy for neuroblastoma infants. In the present paper, retrospective considerations and current stategy for neuroblastoma infants in Japan will be discussed.
Chemotherapy, Adjuvant ; Humans ; Incidence ; Infant* ; Japan* ; Mass Screening ; Neuroblastoma* ; Population Characteristics ; Prognosis ; Retrospective Studies

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