Résumé
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.
Sujets)
Enfant , Femelle , Humains , Mâle , Cause de décès , Protocoles cliniques , Cytogénétique , Dacarbazine , Diagnostic , Survie sans rechute , Épidémiologie , Hémorragie , Incidence , Estimation de Kaplan-Meier , Corée , Leucémies , Dossiers médicaux , Pneumopathie infectieuse , Leucémie-lymphome lymphoblastique à précurseurs B et T , Précurseurs lymphoïdes B , Récidive , Études rétrospectives , Sepsie , Résultat thérapeutiqueRésumé
Isolated chloromas(granulocytic sarcomas) are rare tumors. Chloromas are masses composed of immature granulocytic cells. Granulocytic sarcoma occurs primarily in patients with acute myelogenous leukemia as well as in patients with other myeloproliferative disorders, but rarely in patients with acute lymphoblastic leukemia(ALL). We now describe one patient affected by ALL with isolated granulocytic sarcoma as initial CNS relapses. These unusual clinical manifestation and radiological finding in acute lymphoblastic leukemia should be considered as recurrence of leukemia. Early detection and antileukemic treatment of granulocytic sarcoma are necessarily important for favorable prognosis.
Sujets)
Humains , Leucémies , Leucémie aigüe myéloïde , Syndromes myéloprolifératifs , Leucémie-lymphome lymphoblastique à précurseurs B et T , Pronostic , Récidive , Sarcome myéloïdeRésumé
PURPOSE: von Willebrand disease is a common inherited bleeding disorder characterized by high degree of variable clinical presentation due to either quantitative or qualitative defects in von Willebrand factor. Its incidence in Korea is not well studied while that in western countries is extensively studied. METHODS: We classified 16 cases of vWD from 14 unrelated families based on vWF antigen, ristocetin cofactor activity, factor VIII activity and vWF multimeric patterns analysed by agarose gel electrophoresis, according to a revised classification by ISTH. RESULTS: There were 12 cases (75%) of type 1 vWD or 2M/2N with normal multimeric pattern, 3 cases (18.75%) of type 2 vWD lacking high molecular weight multimers and only 1 case of type 3 vWD with no multimers. CONCLUSION: The proportion of each vWD subtype in Korea is similar to that in western countries, however, accurate diagnosis based on ristocetin induced platelet aggregation test, factor VIII binding assay and molecular genetic diagnosis seems to be necessary for a more complete classification of vWD.
Sujets)
Humains , Classification , Diagnostic , Électrophorèse sur gel d'agar , Facteur VIII , Hémorragie , Incidence , Corée , Biologie moléculaire , Masse moléculaire , Agrégation plaquettaire , Ristocétine , Maladie de von Willebrand de type 3 , Maladies de von Willebrand , Facteur de von WillebrandRésumé
PURPOSE: Interleukin-2 (IL-2) exerts anti-cancer effect by increasing NK cell activity when the tumor burden is low. Earlier study conducted with high dose intravenous IL-2 exhibited significant toxicities such as capillary leak syndrome, fever, rash, etc. This study was designed to study the effect of low dose IL-2 in children after autologous PBSCT when the cancer is at minimal level. METHODS: A total of 12 patients (6 AML, 6 NBL) were enrolled in this study from May 1997 to Oct 1999. The age of the patients was between 0.9~15 yr (Median age: 4.35 yr). The AML patients were treated with AML-BFM-87 (5 cases) or CCG-2891 (1 case) protocol, and all the patients underwent autologous PBSCT at CR1. The NBL patients were treated with CCG-3891 (4 cases) or '6 in 1' (2 cases) protocol, and they had operation for residual tumor before PBSCT. The conditioning regimen for AML patients was busulfan (16 mg/kg) and cyclophosphamide (120 mg/kg) (4 cases) or BCVAC (2 cases), while NBL patients were conditioned with carboplatin (1200 mg/m2), etoposide (800 mg/m2) and melphalan (180 mg/m2). Infused stem cell dose was MNC: (4.5+/-1.7) 108/kg, CD34 : (8.6+/-3.2) 106/kg. IL-2 (Proleukin , Chiron) was started subcutaneously after neutrophil engraftment (ANC<500/mm3) with the dose of 3~5 MU/m2 for the first 2 days, 1MU/m2 for the subsequent 12 days, then followed by 14 days of rest. IL-2 was restarted with the same regimen for more than 6 cycles as outpatient. The CBC, total eosinophil count (TEC) and T lymphocyte subsets were checked before and after IL-2 therapy. RESULTS: The mean neutrophil engraftment was achieved on 12.0+/-3.4 days, and mean platelet recovery to more than 50,000/mm3 was achieved on 23.7+/-10.3 days. Common toxicities associated with IL-2 were fever and mild tenderness on injection site, but there was no need to discontinue IL-2. A total of 75 cycles of IL-2 therapy was given. During follow-up for 8~30 months (median 21 months), only 1 relapse occurred until now (neuroblastoma stage IV). All parameters of T lymphocyte subsets increased after IL-2 therapy. TEC increased in mean value after IL-2 and it was statistically significant (P<0.05). The absolute count of CD4 and CD8 was significantly increased (CD4 : 410 to 640, P<0.005, CD8 : 720 to 980, P<0.05). CD4/CD8 ratio remained reversed (<1) throughout the course of IL-2 in most patients. The total NK cell count was increased from 510 to 820 (P<0.005). CONCLUSION: Low dose IL-2 therapy was well tolerated as OPD basis and there was a significant change in T lymphocyte subsets, especially in NK cell count. Even though the follow up duration was short, the high relapse free survival indicates the beneficial effect of low dose IL-2. In the setting of low tumor burden, such as after autologous PBSCT, low dose subcutaneous IL-2 seems to provide effective anti-cancer effect.
Sujets)
Enfant , Humains , Plaquettes , Busulfan , Syndrome de fuite capillaire , Carboplatine , Cyclophosphamide , Traitement médicamenteux , Granulocytes éosinophiles , Étoposide , Exanthème , Fièvre , Études de suivi , Interleukine-2 , Cellules tueuses naturelles , Leucémie aigüe myéloïde , Melphalan , Maladie résiduelle , Neuroblastome , Granulocytes neutrophiles , Patients en consultation externe , Récidive , Cellules souches , Sous-populations de lymphocytes T , Charge tumoraleRésumé
As leiomyosarcomas of smooth muscle origin are rare tumors found in adults, they are even more uncommon among children. Therefore, most of the existing literatures concerning pediatric leiomyosarcomas has been limited to case reports. More recently, ultrastructural and immunohistochemical studies revealed that some tumors may have properties more suggestive of nerve cells or vascular endothelial cells, rendering the gastrointestinal stromal tumor a more popular term. We experienced a 13 year old boy who was diagnosed and treated as iron deficiency anemia. Even after iron therapy, he had persistent anemia and endoscopic examination revealed a huge mass in the gastric cardia. Proximal gastrectomy was performed with margins free of tumor. The tumor cells showed high mitotic activity suggesting malignant nature. The immunohistochemical staining was positive for smooth muscle actin while negative for S-100 protein. Brief review of the literatures are presented.
Sujets)
Adolescent , Adulte , Enfant , Humains , Mâle , Actines , Anémie , Anémie par carence en fer , Cardia , Cellules endothéliales , Gastrectomie , Tumeurs stromales gastro-intestinales , Fer , Léiomyosarcome , Muscles lisses , Neurones , Protéines S100Résumé
A 10-year-old male patient affected by type 2 von Willebrand disease (vWD) and his family members were investigated by hemostatic and molecular genetic studies. The propositus, who experienced frequent bleeding episodes, was characterized by a normal level of von Willebrand factor (vWF) antigen (54%), reduced vWF ristocetin cofactor activity (5%), decreased factor VIII clotting activity (25%) and absent high molecular weight multimers in the plasma. An exon 28 fragment coding for the A1 and A2 domains was amplified by polymerase chain reaction and sequenced. We found a heterozygous mutation (G4022A), producing an additional PstI restriction site, which resulted in the substitution of Arg578Gln. Family studies, including the parents and a brother, were negative for this mutation and vWF abnormalities were not observed. We confirmed that G to A mutation in the region of the platelet glycoprotein Ib binding domain of vWF causes the qualitative type 2 defect in von Willebrand disease.
Sujets)
Enfant , Humains , Mâle , Alanine/génétique , Glycine/génétique , Mutation ponctuelle , Maladies de von Willebrand/génétique , Facteur de von Willebrand/génétiqueRésumé
Rifampin is sometimes associated with hematologic complications such as hemolytic anemia or thrombocytopenia. Patients with drug-induced thrombocytopenia develop a drug- dependent antibody that binds to platelets in the presence of the drug causing platelet clearance. It has been previously proposed that the antibody binds the drug, resulting in an immune complex that is then adsorbed onto platelets. However, it has been recently known that drug-dependent antibodies bind to one or more of the platelet membrane glycoprotein Ib, IIb, IIIa, and IX. We, hereby, report a case of rifampin-induced thrombocytopenia in which drug-dependent antibody specific for platelet glycoprotein Ib/IX and IIb/IIIa was demonstrated by modified antigen capture ELISA method. The case was a 37 year-old female who had had pulmonary tuberculosis and taken antituberculous regimen including rifampin. Intermittent epistaxis appeared 10 days after treatment with rifampin. She was admitted to hospital due to gingival bleeding for 3 days and menorrhagia for 2 days. On admission, her platelet count was dropped to 7,000/microliter.
Sujets)
Adulte , Femelle , Humains , Anémie hémolytique , Anticorps , Complexe antigène-anticorps , Plaquettes , Test ELISA , Épistaxis , Glycoprotéines , Hémorragie , Ménorragie , Numération des plaquettes , Complexe glycoprotéique GPIb-IX plaquettaire , Rifampicine , Thrombopénie , Tuberculose pulmonaireRésumé
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common hereditary enzyme disorder and more than 200 million people have a deficiency in this enzyme. It is a globally important cause of neonatal jaundice and causes life-threatening hemolytic crisis in childhood. At later ages, certain drugs such as antimalarials, and fava beans cause hemolysis among G6PD deficiency patients. The frequency and severity is influenced by genetic and cultural factors. It is common in Mediterranean, African, and some East Asian populations but rare in Korea. Four cases of G6PD deficiency which were first noticed in Korea are investigated with their clinical features.
Sujets)
Enfant , Enfant d'âge préscolaire , Humains , Mâle , Déficit en glucose-6-phosphate-déshydrogénase/diagnostic , PedigreeRésumé
No abstract available.
Sujets)
Plaquettes , Électrophorèse sur gel de polyacrylamide , Glycoprotéines membranaires , Membranes , ThrombasthénieRésumé
Hodgkin's disease, manifested as a second malignant neoplasm in acute lymphoblastic leukemia, rarely occurs, with seventeen cases reported including this cases. We presented the clinical and pathological features of a nine-year-old male child with acute lymphoblastic leukemia in remission. He had cervical lymph node involvement 22 months after the diagnosis of leukemia as an initial presentation of Hodgkin's disease of mixed cellularity. A brief review of related literatures was also done.
Sujets)
Enfant , Humains , Mâle , Antinéoplasiques/administration et posologie , Maladie de Hodgkin/complications , Leucémie-lymphome lymphoblastique à précurseurs B et T/complications , Seconde tumeur primitive/anatomopathologieRésumé
No abstract available.
Résumé
To determine laboratory evidence suggesting immunological abnormalities in persons with hemophilia, we evaluated the immunological status of 75 Korean hemophiliacs, seronegative for human immunodeficiency virus (HIV) antibodies, who have been treated only with Korean factor VIII concentrates. From this study, it was shown that Korean hemophiliacs had decreased CD4 levels, increased CD8 levels, and decreased CD4:CD8 ratios. Diminished lymphocyte response to the mitogens, phytohemagglutinin and concanavalin A, and decreased natural killer cell activity were observed in the hemophiliacs. In addition, production of interleukin-II in the hemophiliacs was lower than in the healthy controls. The percentage of B lymphocytes was significantly reduced but the serum levels of immunoglobulin (Ig) G were elevated. However, the serum Ig A and Ig M levels were normal. This study demonstrated a high frequency of immunological abnormalities in HIV antibody negative Korean hemophiliacs treated only with domestic factor VIII concentrates.