No abstract available.
Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells*

No abstract available.
Child* ; Down Syndrome* ; Humans

No abstract available.
Klinefelter Syndrome*

No abstract available.
Turner Syndrome*

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

No abstract available.
Chondroma* ; Mediastinum*

No abstract available.
Leukemia*

BACKGROUND: The aim of this study is to evaluate the therapeutic effect of splenectomy in children with chronic idiopathic thrombocytopenic purpura(ITP) who do not respond to corticosteroids and intravenous immunoglobulin(IVIG). METHODS: Fifteen patients(7 boys and 8 girls) who were admitted to St. Mary's Hospital under the diagnosis of ITP from December 1990 to February 1997 were enrolled In this study. All had chronic forms(more than 6 months after diagnosis) and were refractory to corticosteroids(prednisolone 1~2mg/kg) and IVIG(400mg/kg for 5 days or 1 g/kg for 2 days). We you-finely employ pneumococcal vaccine in 13 patients before splenectomy slnce 1992. Antiplatelet antibody was examed in 10 patients. We measured platelet count at preoperative time, postoperative 1 day, 1 month, and 3 months or more. Hematologic results were analyzed according to Berchtold and McMillan's criteria (1) complete response(CR) >120 x 10(9)/l; (2) partial response(PR) >50 x 10(9)/l (3) no response(NR) < 50 x 10(9)/l. RESULTS: The median age at operation was 10 yr(6~16). The median time interval between diagnosis and splenectomy was 2 yr 2 mo(6 mo~5 yr 5 mo). Antiplatelet antibody was positive in 2 cases of 10 cases(20%). Of 15 cases, 12 cases were corticosteroid resistant and 3 cases were corticosteroid dependent. The postsplenectomy platelet count(median 407 x 10(9)/l of 1 day; 254 x 10(9)/l of 1 month; 227 x 10(9)/l of 3 months or more) was significantly higher than presplenectomy platelet count(median 9 x 10(9)/l)(P<0.05). All patients showed complete or parial response throughout the follow up period. Accessory spleen was found in 1 case and removed during operation. There were no serious complications following splenectomy except mild fever in 2 cases(14%). CONCLUSION: Splenectomy appears to be an effective and relatively safe treatment for patients with chronic ITP who have had inadequate response to conventional therapies.
Adrenal Cortex Hormones ; Blood Platelets ; Child ; Diagnosis ; Fever ; Follow-Up Studies ; Humans ; Platelet Count ; Purpura, Thrombocytopenic* ; Spleen ; Splenectomy*

PURPOSE: We reviewed the result of allogeneic bone marrow transplantation(BMT) from HLA-identical sibling donors in children with refractory stem cell disorder along with future implication. METHODS: Forty-two children with refractory stem cell disorder received BMT from HLA-identical sibling donors between Nov. 1983 and Feb. 1995. Out of 42 children, 23 cases were severe aplastic anemia(SAA) and 19 cases were refractory leukemias. There were 20 male and 22 female with median age of 13 years (range, 2-17) and median follow-up of 36 months (range, 4-139 months). RESULTS: 1) The overall survival rate of all patients was 73.8%. The survival rate for SAA cases was 87.0%, while that for leukemia was 57.9%. 2) Acute GVHD(> or = grade II) was observed in 16.7% of all patients, 8.7% of SAA patients and 26.3% of leukemia patients, respectively. Chronic GVHD developed in 9.5% of all patients, 4.8% of limited type and 4.8% of extended type. No death was directly attributable to GVHD. 3) The causes of death after allogeneic BMT were graft rejection(7.1%), relapse of leukemia(7.1%), thrombotic thrombocytopenic purpura(4.8%), veno-occlusive disease, sepsis and CMV pneumonia respectively 2.4%. 4) The most common complication except death after allogeneic BMT was herpes zoster(26.2%). The other complications were hemorrhagic cystitis(7.1%), bronchiolitis obliterans and measles respectively 2.4%. CONCLUSIONS: We confirmed that allogeneic BMT is the curable treatment for children with refractory stem cell disorder. The most important factors that influence the result of transplantation are interval between diagnosis and transplantation in severe aplastic anemia and remission state at transplantation in leukemia.
Anemia, Aplastic* ; Bone Marrow Transplantation* ; Bone Marrow* ; Bronchiolitis Obliterans ; Cause of Death ; Child* ; Diagnosis ; Female ; Follow-Up Studies ; Humans ; Leukemia* ; Male ; Measles ; Pneumonia ; Recurrence ; Sepsis ; Siblings ; Stem Cells ; Survival Rate ; Tissue Donors ; Transplants

Aplastic anemia is a rare disease, which is characterized by pancytopenia and hypocellular bone marrow without infiltration of abnormal cells or fibrosis. The incidence in Asia is higher than in the West and new cases are diagnosed at a rate of 5.1 per million pediatric populations per year in Korea. The pathophysiology is understood roughly by defective hematopoiesis, impaired bone marrow microenvironment and immune mechanism. Treatments are performed on basis of pathogenesis and selected depending on the severity. Immunosuppressive therapy with antilymphocyte or antithymocyte globulin and cyclosporine is effective in the majority of patients but has some problems including relapse or clonal evolution. Recently, there have been clinical trials of immunosuppression with hematopoietic growth factors or other drugs. Allogeneic hematopoietic stem cell transplantation (HSCT) is curative in children with severe aplastic anemia. The overall survival in HSCT from HLA-identical sibling is higher than alternative donor, including HLA matched unrelated donor or cord blood. We have to consider quality of life after HSCT because of high survival rate. However, chronic graft versus host disease and graft failure are important factors that affect the quality of life and overall survival. We need further investigation to make new regimens aimed at overcoming these risk factors and perform clinical trials.
Anemia, Aplastic* ; Antilymphocyte Serum ; Asia ; Bone Marrow ; Child ; Clonal Evolution ; Cyclosporine ; Fetal Blood ; Fibrosis ; Graft vs Host Disease ; Hematopoiesis ; Hematopoietic Stem Cell Transplantation ; Humans ; Immunosuppression ; Incidence ; Intercellular Signaling Peptides and Proteins ; Korea ; Pancytopenia ; Quality of Life ; Rare Diseases ; Recurrence ; Risk Factors ; Siblings ; Survival Rate ; Tissue Donors ; Transplants ; Unrelated Donors