The t(3;21) (q26;q22) is associated with chronic myelogenous leukemia in blast crisis, leukemia evolving from therapy-related myelodysplasia, and with leukemia following other hematopoietic proliferative diseases. The t(3;21) is rare secondary aberration in blast crisis of Philadelphia(Ph)-positive chronic myeloid leukemia, which may be restricted to patients entering myeloid blast crisis. We report here in one case of chronic myeloid leukemia in blast crisis which reveals both t(9;22) (q34;q11), and t(3;21) (q26 ;q22). A 62-year-old male was diagnosed as chronic myeloid leukemia 5 years ago, received hydroxyurea therapy, and admitted because of gingival bleeding and fever. On examination, splenomegaly and leukocytosis with proliferated blasts(91%) in peripheral blood were noted. Bone marrow aspirate showed hypercellularity with severe blast proliferation(92.5%) which revealed all negative in peroxidase and PAS stain. Cytogenetic study of bone marrow cells showed the karyotype 46, XY, t(3;21) (q26;q22), t(9;22) (q34;q11), which might be suspected as myeloid blast crisis. Above finding was confirmed by the result of immunophenotyping(CD13 43.6%, CD34 68.2%, HLA-DR 91.6%). He received intensive chemotherapy, but still sustained proliferation of blasts was noted . The follow up cytogenetic study was as follows: 46, XY, 4(3;21) (q26:22), t(9;22) (q34;q11)/46, XY, t(3;21)(q26;q22), del(8) (q22), t(9:22) (q34,q11)/46, XY (16/3/1). He died soon from severe pancytopenia and sepsis.
Blast Crisis* ; Bone Marrow ; Bone Marrow Cells ; Cytogenetics ; Drug Therapy ; Fever ; Follow-Up Studies ; Hemorrhage ; HLA-DR Antigens ; Humans ; Hydroxyurea ; Karyotype ; Leukemia ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive* ; Leukocytosis ; Male ; Middle Aged ; Pancytopenia ; Peroxidase ; Sepsis ; Splenomegaly

BACKGROUND: The combination of Philadelphia chromosome (Ph) and monosomy 7(-7) was rarely observed in acute lymphoblastic leukemia (ALL). With the results from immunophenotyplc and molecular analysis, Philadelphia chromosome positive ALL with monosomy 7[Ph(+)/-7] has been considered that it may be derived from neoplastic transformation at the pluripotent stem cell level. We compared the clini-cal, laboratory, and hematological findings between 5 cases of Ph(+)/-7 and 5 cases of Ph(+) without monosomy 7 [Ph (+) /N7]. METHODS: During the period from January, 1995 to December, 1996, total 72 cases of ALL were confirmed among 259 cases of hematologic malignancy with bone marrow cytogenetic analysis. Among 72 ALL cases, 5 cases of Ph(+)/-7(monosomy 7 or 7q abnormalities) were compared with Ph only or Ph without monosomy 7(ph(+)/N7] on the hematological, immunophenotypic, other laboratory, clinical findings and event ree survival (EFS) The karyotyping of the bone marrow specimens was analysed byshort-term unsynchronized culture methods such as overnight colcemid treatment and 24 hours incubation following ethidium bromide treatment. RESULTS: The mean age of Ph(+)/-7 was 30.6+/-12.8 years, and it was significantly different from that of Ph(+)/N7 (p=0.009), Four cases of Ph(+)/-7 were classified as ALL L2 subtype, and 2 cases revealed CNS involvements. Immunophenotyping was positive in CD10, CDl9, CD2O, CD22 and HLA-DR. But one case revealed e-B-lymphoid lineage with positivity in CD34, CDl3, and CD33. The response to chemotherapy and EFS was very poor in Ph(+)/-7 group, and the mean EFS was 3.2+/-1.9 months(p=0.014). All of cases showed induction on failure in chemotherapy, relapsed with bone marrow, CNS and extramedullary involvements, and expired due to sepsis. CONCLUSIONS: Ph(+)/-7 ALL had very Poor clinical course with being resistant to chemotherapy and unfavorable prognosis, revealed L2 subtype by FAB classification, and was slightly older in ages compared with Ph(+)/N7 ALL.
Bone Marrow ; Classification ; Cytogenetic Analysis ; Demecolcine ; Drug Therapy ; Ethidium ; Hematologic Neoplasms ; HLA-DR Antigens ; Hydrogen-Ion Concentration ; Immunophenotyping ; Karyotyping ; Monosomy* ; Philadelphia Chromosome* ; Pluripotent Stem Cells ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Prognosis ; Sepsis

Vibrio metschnikovii is worldwidely distributed in the aquatic environment and human infections are very rarely associated, such as septicemia, urinary tract infection, wound infection, and peritonitis. V. metschnikovii is negative in nitrate reduction and oxidase reaction, and these findings are different from other vibrio species. V. metschnikovii was isolated from the ascitic fluid and blood of a patient with peritonitis, sepsis and renal insufficiency. This patient was a 41-year old man who suffered from post-necrotic liver cirrhosis, chronic hepatitis B, gastric ulcer, esophageal varix bleeding, and alcoholism. He had neither history of ingestion of seafoods nor exposure to seawater before onset of illness. He was successfully treated with antimicrobial agents. This is the first case report of septicemia and peritonitis by V. metschnikovii in Korea.
Adult ; Alcoholism ; Anti-Infective Agents ; Ascitic Fluid ; Eating ; Esophageal and Gastric Varices ; Hemorrhage ; Hepatitis B, Chronic ; Humans ; Korea ; Liver Cirrhosis ; Oxidoreductases ; Peritonitis* ; Renal Insufficiency ; Seafood ; Seawater ; Sepsis* ; Stomach Ulcer ; Urinary Tract Infections ; Vibrio* ; Wound Infection

No abstract available.
Chromosome Aberrations*

No abstract available.
Blood Coagulation Factors* ; Fibrinogen*

No abstract available.
Blood Coagulation Factors* ; Fibrinogen*

BACKGROUND: In spite of appropriate therapy and control for tuberculosis, the prevalence of tuberculosis is still frequent in Korea. Emerging infection and rapid detection of multidrug-resistant Mycobacterium tuberculosis (MDR-TB) are major interests in microbiologic laboratories. In this study, we evaluated the usefulness of random amplified polymorphic DNA (RAPD) genotyping for molecular epidemiological characteristics of MDR-TB. METHODS: We analyzed 64 clinical strains of M. tuberculosis including 35 strains which showed resistance to one or more antimycobacterial drugs and M. tuberculosis H37Rv (ATCC 27294), as a drug-sensitive control strain. RAPD genotyping analysis was carried out under eight reaction conditions and using ten random primers (A-1245, AP-50, B-1245, DKU-44, DKU-49, Leg-1, INS-2, IS-986-FP, PF-15 and MBR). RESULTS: RAPD patterns using six primers (IS-986-FP, DKU-44, DKU-49, INS-2, B-1245, and AP-50) showed marked polymorphisms that were easier to discriminate than those with other primers. RAPD patterns represented various polymorphisms among 64 strains. However, RAPD could not discriminate MDR-TB strains from drug-sensitive ones. CONCLUSIONS: RAPD genotyping is assumed a preferable technique for discrimination among clinical strains of M. tuberculosis but not for specifying MDR-TB strains.
Discrimination (Psychology) ; DNA* ; Korea ; Mycobacterium tuberculosis* ; Mycobacterium* ; Prevalence ; Tuberculosis

No abstract available.
Blood Platelets* ; Humans ; Platelet Aggregation* ; Plateletpheresis* ; Tissue Donors*

During the period of 4 years from May, 1981 to April, 1985, 71 cases of obstructing subinfundibular muscular bundles of the right ventricle in congential cardiovascular anomalies were diagnosed by cariac catheterization and angiography at Hanyang University. The finding of right ventricular angiogram and degree of the right ventricular outflow obstruction, clinical data, electrocardiographic data and hemodynamic data were correlated irrespectively. The summary of this article is as follows. 1) The age of patients was ranged from 1 to 22 years old with a mean age of 8 years old. There were 39 males and 32 females with M:F ratio of 1.2:1. The incidence of obstructing subinfundibular muscular bundles of the right ventricle in congenital cardiovascular anomalies was higher with increasing age. 2) The associated cardic anomalies were as follows: 40 cases (56%) of isolated ventricular septal defect(VSD), 13(18%) of tetralogy of Fallot physiology, 7(10%) of patent ductus arteriosus(PDA), 3(4%) of pulmonary stenosis, 1 aortic stenosis, 1 double outlet of right ventricle(DORV), 1 trilogy, 1 ostium secundum defect, etc. The incidence of VSD with or without other associated cardiovascular anomalies was 56 cases(79%) out of 71 cases. 3) Maximum systolic pressure gradient between proximal and distal chamber of the right ventricle were under 25 mmHg in 32 cases, between 25 and 50 mmHg in 13 and above 50 mmHg in 26. Pressure gradients of all 7 cases with PDA were under 25 mmHg. 4) Correlative assesment of angiographic manifestation(2 indicies:Diameter of right ventricular outflow tract(systolic phase)/diameter of tricuspid valvular annulus(diastolic phase)=OT/TV, Diameter of right ventricular outflow tract(systolic phase)/Length of right ventricular diaphragmatic surface(systolic phase)=OT/RV) according to pressure gradient, OT/Tv and OT/RV values were lower the increasing pressure gradient, between proximal and distal chamber of the right ventricle. These were reverse correlations but coefficients of correlation(r) were-0.49and -0.48. Therefore, the degree of right ventricular outflow obstruction could be predicted, using 2 indices of right ventricular angiogram in individual cases, but could not be calculated accurately. 5) This indicated that pressuer gradient was also affected by technical errors, variable cardic anomalies, development of sinusoid, age and the other factors. 6) We assumed that non-invasive Doppler echocardiography could be useful in making the diagnosis and follow up of the patient with obstructing subinfundibular muscle bundles in right ventricle.
Angiography ; Aortic Valve Stenosis ; Blood Pressure ; Catheterization ; Catheters ; Child ; Diagnosis ; Echocardiography, Doppler ; Electrocardiography ; Equidae ; Female ; Heart Ventricles* ; Hemodynamics ; Humans ; Incidence ; Male ; Physiology ; Pulmonary Valve Stenosis ; Tetralogy of Fallot ; Ventricular Outflow Obstruction ; Young Adult

Ganglioneruroblastoma and neuroblastoma are among commonest types of childhood malignancy and a number of unique paraneoplastic syndromes have associated with both localized and disseminated neuroblastoma. The coincidence of neuroblastoma and myoclonic encephalopathy or other paraneoplastic syndromes occurs relatively rare, and therefore, failure to recognize this association could result in delays in both diagnosis and treatment, and the result could prove to be unfortunately fatal. The mechanism which underlies the remote damaging effect of neural crest tumor, especially neuroblastoma, on the nervous system resulting in myoclonic encephalopathy is by no means clear. In addition the nature and the extent of the pathologic lesion are inconsistent. We experienced a case of myoclonic encephalopathy associated with an occult mediastinal ganglioneuroblastoma in a 22-month-old girl who was hospitalized for inability to walk without support and tilting of the head to the left side. She became increasingly ataxic, and during the hospitalization myoclonic jerks of upper extremities and head along with chaotic, rapidly flickering, multidirectional spontaneous eye movements, were noted. Laboratory data included normal complete blood count, urinalysis, BUN and creatinine, electrolytes and bone marrow. Chest X-ray and chest CT revealed a relatively well marginated right posterior mediastinal mass. In a 24 hours urine excretion test, VMA and catecholamines were increased. Over the next 2 weeks, a surgical exploration revealed a right posterior mediastinal mass. Microscopically the mass proved to be a ganglioneuroblastoma, extending to right innominate artery and right axillary lymph nodes. Within 2 weeks after the surgery, radiotherapy (2,400 rads) and chemotherapy (CTX, DTIC, VCR) were started, but corticosteroid was not used. She has been free of tumor and abnormal neurological systemic symptoms and signs for 1 1/2 year since the completion of chemotherapy. In the 3 1/2 years follow-up period, her neurologic symptoms has completely resolved by the completion of 2 years chemotherapy. We report a case of mycoclonic encephalopathy associated with hidden ganglioneuroblastoma in 22-month-old girl.
Blood Cell Count ; Bone Marrow ; Brachiocephalic Trunk ; Catecholamines ; Creatinine ; Dacarbazine ; Diagnosis ; Drug Therapy ; Electrolytes ; Epilepsies, Myoclonic* ; Eye Movements ; Female ; Follow-Up Studies ; Ganglioneuroblastoma* ; Head ; Hospitalization ; Humans ; Infant ; Lymph Nodes ; Myoclonus ; Nervous System ; Neural Crest ; Neuroblastoma ; Neurologic Manifestations ; Paraneoplastic Syndromes* ; Radiotherapy ; Thorax ; Tomography, X-Ray Computed ; Upper Extremity ; Urinalysis