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

No abstract available.
Chromosome Aberrations*

No abstract available.
Blood Coagulation Factors* ; Fibrinogen*

No abstract available.
Blood Coagulation Factors* ; Fibrinogen*

No abstract available.
Leukemia, Promyelocytic, Acute*

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

No abstract available.
Cholestasis, Intrahepatic*

Congenital adrenal hyperplasia is inherited disorder of adrenal steroidogenesis. 21-hydroxylase deficiency is the most commone enzymatic defect and is divided into classic and late-onset or nonclassic forms. Both classic non-classic 21-hydrozylase deficiencies are inherited in a recessive manner as allelic variants. But it is rare that happened in twin infants. Chief complaints of affected twins in our case were ambiguous genitalia, hyperpigmentation and dehydrations. They were revealed into hyponatremia, hyperkalemia and increased amount of serum progesterone, 17-hydroxyprogesterone and urinary 17-ketosteroid excretion and were administered with DOCA, 9alpha-fluorohydrocortisone, hydrocortisone to control the electrolyte imbalance. And now, both of them are going to normal ratio of weight gain and body growth.
17-alpha-Hydroxyprogesterone ; Adrenal Hyperplasia, Congenital* ; Desoxycorticosterone Acetate ; Disorders of Sex Development ; Humans ; Hydrocortisone ; Hyperkalemia ; Hyperpigmentation ; Hyponatremia ; Infant ; Progesterone ; Steroid 21-Hydroxylase* ; Twins* ; Weight Gain

No abstract available.
Tuberculosis, Pulmonary*

No abstract available.
Carcinoma, Squamous Cell* ; Cisplatin* ; Drug Therapy, Combination* ; Female ; Ovary* ; Paclitaxel*