BACKGROUND: Although immunophenotyping of leukemias has improved diagnostic accuracy and reproducibility, it has also caused diagnostic confusion regarding the lineage of leukemic cells. So far, lots of papers about acute leukemias with coexpression of another lineage markers with different technical methodologies and different criteria have been published in Korea and other countries. The authors investigated the frequency and immunophenotypic characteristics of the leukemias with aberrant lineage markers from data obtained at Korea University Hospital by a retrospective study. METHODS: From Jan. 1993 to Feb. 1996, 179 leukemias had been requested for immunophenotypig and 28 cases among them with unusual immunophenotypes were retrieved according to their immunophenotyping results. For the final diagnosis all the slides stained with Wright-Giemsa, peroxidase, Periodic-Acid Schiff, Sudan black B, and nonspecific esterase were re-examined, and all the flow cytometric results were reanalyzed. RESULTS: Among 28 cases, 3 cases(10%) were acute biphenotypic leukemias(BP) one with B lymphoid and myeloid markers and the other two with T lymphoid and myeloid markers. One case of intralineage bilinear acute leukemia(ILBL) with two separate populations of megakaryocytic cells and monocytic cells was noted. 6 cases(21%) were acute myeloblastic leukemias expressing lymphoid associated markers(Ly+AMLs; CD19) and 8 cases(28%) were myeloid antigen-positive acute lymphoblastic leukemias(My+ALLs, four with CD13+ and three with CD33+ and one with blastic transformation of chronic myelogeneous leukemia). Because of the change in diagnostic criteria, lymphocyte contamination, or low setting of negative control, 10 cases (36%) were not included to be of unusual immunophenotypes. CONCLUSIONS: Frequency of acute hybrid leukemia was 2.2 % of all leukemias. Ly+AMLs was 3.4%, and My+ALL was 4.4%. In conclusion, first, quality control of the flow cytometry and careful interpretation especially in terms of positive cut-off value and gating, are needed. Secondly, national guidelines for the criteria of the hybrid leukemia and My+ALLs and Ly+AMLs are necessary for the elucidation of the prognostic implication of those leukemias.
Carboxylesterase ; Diagnosis ; Flow Cytometry ; Immunophenotyping ; Korea ; Leukemia* ; Leukemia, Myeloid, Acute ; Lymphocytes ; Peroxidase ; Quality Control ; Retrospective Studies ; Sudan

Malignant mixed mullerian tumor (MMMT) is an unusual tumor composed of malignant epithelial and nonepithelial components in the same lesion and is subdivided into homologous and heterologous types. Epidemiologically, these tumors are associated with prior pelvic irradiation, functioning ovarian lesions, exogenous estrogen therapy and rarely endometriosis. We experienced a case of uterine MMMT which arose from adenomyosis in a 47-year-old woman who had no specific past medical history. The posterior uterine corpus showed a 3.5x3.0x2.0 cm sized, relatively well defined tumor mass within the background of the adenomyosis. The tumor was composed of well differentiated endometrial adenocarcinoma and sarcomatous stroma with foci of rhabdomyosarcomatous differentiation confirmed by immunohistochemical and electron microscopic studies. Through the immunohistochemical study, both the epithelial and nonepithelial components were positive for cytokeratin and it suggested that the sarcomatous area originated from metaplasia of the adenocarcinoma component. From the overall findings, it is regarded as an uterine heterologous MMMT which arose from adenomyosis.
Adenocarcinoma ; Adenomyosis* ; Endometriosis ; Estrogens ; Female ; Humans ; Keratins ; Metaplasia ; Middle Aged ; Rhabdomyosarcoma ; Uterus*

No abstract available.
Rheumatic Diseases* ; Spondylarthropathies

Traumatic intracranial lesion has been one of the most frequent and serous problem in neurosurgical pathology. CT made it possible to get prompt diagnosis and surgical intervention of intracranial lesions by its safety, fastiness and accuracy. Computed tomographic scan was carried out on 1309 cases at Soonchunhyang Chunan Hospitalfor 15 months from Oct. 1983 to Dec. 1984. We have reviewed the computed tomographic scans of 264 patients whichshowed traumatic intracranial lesion. The results were as follows: 1. Head trauma was the most frequentlydiagnosed disase using computed tomographic scans(57.8%), and among 264 cases the most frequent mode of injury wastraffic accident (73.9%). 2. Skull fracture was accompained in frequency of 69.7% and it was detected in CT in38.6%: depression fractue was more easily detected in 81%. 3. Countercoup lesion(9.5%) was usually accompained with temporal and occipital fracture, and it appeared in lower incidence among pediatric group. 4. Intracranial lesions of all 264 cases were generalized cerebral swelling(24.6%), subdural hematoma(22.3%), epiduralhematoma(20.8%), intracerebral hematoma(6.1%), and subarachnoid hemorrhage(3.0%). 5. The shape of hematoma wasusually biconvex(92.7%) in acute epidural hematoma and cresentic(100%) in acute subdural hematoma, but the morechronic the cases became, they showed planoconvex and biconvex shapes. 6. Extra-axial hematoma was getting decreased in density as time gone by. 7. Hematoma density was not in direct proportion to serum hemoglobin levelas single factor.
Chungcheongnam-do ; Craniocerebral Trauma ; Depression ; Diagnosis ; Hematoma ; Hematoma, Subdural, Acute ; Humans ; Incidence ; Pathology ; Skull Fractures

Eight bacterial strains were examined whether they have phoP/phoQ genes which were known to be involved in the intracellular survival of Salmonella typhimurium. The phoP/phoQ operon were known to sense the stimuli of the genes involved in the adaptation of the environment. Using 514-basepairs EcoRV DNA fragment of phoP region of Salmonella typhimurium as a probe, dot blot hybridization were performed. Chromosomal DNAs of Klebsiella pneumonia, Pseudomonas aeruginosa, Serratia marscescens, Enterobacter cloacae, Salmonella typhimurium, Escherichia coli, Shigella dysenteriae, and Listeria monocytogenes were examined by DNA hybridization assay. Against our expectation, intracellular pathogen, L. monocytogenes, did not have similar DNA sequences to phoP/phoQ of S. typhimurium, while E, coli, S. dysenteriae, and E. cloacae showed the positive signal even though they were not intracellular pathogens. This result suggested that the phoP/phoQ operon was absent in intracellular pathogenic bacterias other than S. typhimurium. Rather it was found in phylogenetically closer bacterias to S. typhimurium, which were not able to survive in intracellular environment. Some different mechanism, which is not dependent on phoP/phoQ operon, could be involved in the intracellular survival of L. monocytogenes.
Bacteria* ; Base Sequence ; Cloaca ; DNA ; Enterobacter cloacae ; Escherichia coli ; Klebsiella ; Listeria monocytogenes ; Operon ; Pneumonia ; Pseudomonas aeruginosa ; Salmonella typhimurium ; Serratia ; Shigella dysenteriae

No abstract available.
Child ; Child, Hospitalized* ; Humans ; Hypertension*

Porencephaly is relatively rare condition defined by an defect or a defect or cavity in the cerebrum owing to a developmental malformation or to a destructive lesion. Fory-five porencephaly patients diagnosed by Brain CT were clinically analyzed and the following results were obtained. 1) By the age group presenting initial symptoms, the peak incidence was from 1 month to below 3 years old. 2) In initial symptoms, seizure, spastic weakness, headache were showed in order of frequency. But 7 cases (15.5%) were asymptomatic. 3) The latency of diagnosis after presenting initial symptoms from the symptom onset time to 10 years. 4) The subsequent symptoms were as follows: spastic weakness, speech disturbance, gait disturbance, mental retardation, sensory loss and seizure showed independently or combined. 5) As etiologic factor, 21 cases (46.7%) were congenital, 16 cases (35.5%) were post-traumatic or post-operative and 8 cases (17.8%) were perinatal. 6) The prognosis was seen various from mild to severe. Out of 45 cases, 29 cases (64.4%) were no complications. But the prognosis in patients with post-traumatic or postoperative etiological factors was poor. With the advent of brain CT and the resultant capability of detecting structural defect and cerebral lesions responsible for epilepsy or focal neurologic signs, porencephaly was seen to be readily recongizable by CT examination. Since porencephaly is a significant contributor to the spectrum of CNS lesion and benign condition, ist recognition is important in determining prognosis and therapy.
Brain ; Cerebrum ; Child, Preschool ; Diagnosis ; Epilepsy ; Gait ; Headache ; Humans ; Incidence ; Intellectual Disability ; Muscle Spasticity ; Neurologic Manifestations ; Prognosis ; Seizures

Bullous pemphigoid(BP) and herpes gestationis(HG) are subepidermal bullous diseases which show clinical and immunological similarities. Both diseases show immune deposits along the basement membrane zone and their autoantibodies bind a common antigenic site within the non-collagenous stretch of the 180 kDa BPAG2 ectodomain. Besides its association with pregnancy, HG has some characteristic features that distinguish it from BP. The serum of patients with HG often contains an IgG that avidly fixes complement, and showes IgG1 subclass predominance. We report here two cases of non-pregnant young women presenting clinical and histological features of bullous pemphigoid or herpes gestationis. The immunopathology, IgG subtyping and immunoblotting studies showed that the autoantibodies in the patients were the characteristic ones of herpes gestationis. The patients might be a subtype of BP that have characteristics of autoantibodies of HG patients.
Autoantibodies* ; Basement Membrane ; Complement System Proteins ; Female ; Humans ; Immunoblotting ; Immunoglobulin G ; Pemphigoid Gestationis* ; Pemphigoid, Bullous* ; Pregnancy

OBJECTIVES: Anticardiolipin antibody (ACA) and lupus anticoagulant (LA) are acquired antiphospholipid antibodies (APAs), which are regarded as important risk factors far vascular thrombosis and recurrent fetal loss. Although the clinical relevance of APAs in dialysis patients is uncertain, recent studies have suggested that APAs are involved in bioincompatibility and thrombogenic complications in hemadialysis (HD) patients. METHOD: We performed a cross sectional study of ACA and LA in 50 stable HD patients and their 68 vascular accesses (52 native arteriovenous fistulae and 16 synthetic arterovenous grafts), with the analysis of factors associated with the presence of APAs and the retrospective evaluation of vascular access occlusion (VAO). LA was assessed by platelet neutralization method whereas IgG-ACA was measured by a solid phase ELISA. Values higher than 23GPLU/ml (IgG phospholipid units) were considered to be positive for IgG-ACA and positive values for LA was more than 8 seconds in prolongation of the clotting time with human platelet lysate. Vascular access survival was assessed by Kaplan- Meier method, RESULTS: The mean age of the subject (M:F 21:29) was 46 years and the mean duration of hemodialysis was 49 months. The frequency of VAO in entire subjects was 0.45+/-0.98 episodes/patient year. The median value of IgG-ACA was 16.0 GPLU/ml with a distribution from 2.7 to 46.1GPLU/ ml. The median titer of I.A was 4.5 (3.1-45.6) seconds. Fourteen patients (28%) were found to have at least one episode of VAO. In spite of comparable clinical and biochemical data according to the presence of VAO, the titers of IgG-ACA (13.6+/-7.7 vs, 20.3+/-8.7GPLIJ/ml, P<0.05) and LA (4.5+/-2.9 vs. 11.7 +/-12.6sec, P<0.05) were significantly higher in VAO group. Six out of 50 patients(12%) had an increased titer of IgG-ACA and LA was found in 11 patients(22%). No patients were positive for ACA and LA simultaneously. There was no significant difference in sex, etiology of ESRD, diabetic status, the dosage of heparin during HD or the amount of erythropoietin administered according to the presence of APAs. We could not find any significant correlation between the titer of APAs and age, duration of dialysis, blood pressure, platelet count and biochemical parameters. In the patients with positive ACA, the frequency of VAO was 1.05+/-0.12 episodes/patient year, which was significantly higher than patients without ACA (0.33+/-0.17 episodes/ patient year, P<0.05). In the patients with the presence of LA(1.06+/-0.43 vs. 0.12+/-0.06 episodes/ patients year, P<0.01). The median vascular access survival time in IgG-ACA positive patients (32.7 months) was significantly decreased compared to 66.8 months in IgG-ACA negative group. CONCLUSION: Our data suggest that the presence of APAs (ACA and/or LA) affects the event-free vascular access survival in HD patients. Therefore the evaluation of APAs status have to be included in the diagnostic strategies for the patients with recurrent VAO. Further studies are necessary to explore the pharmacologic intervention method to decrease APAs and prevent VAO in HD patients.
Antibodies, Anticardiolipin* ; Antibodies, Antiphospholipid ; Arteriovenous Fistula ; Blood Platelets ; Blood Pressure ; Dialysis ; Enzyme-Linked Immunosorbent Assay ; Erythropoietin ; Heparin ; Humans ; Kidney Failure, Chronic ; Lupus Coagulation Inhibitor* ; Platelet Count ; Renal Dialysis* ; Retrospective Studies ; Risk Factors ; Thrombosis

Several observation suggest that the antimelanocyte autoantibodies could play a role in melanocyte destruction. Some experiments indicate that melanocyte antibodies from patients with vitiligo can kill melanocyte in vitro. In these experiments, we demonstrated that vitiligo patient's sera containing antimelanocyte antibodies can lyse cultured human melanocytes by complement activation. Melanocyte cytotoxicity was measured using the ethidium bromide/ acridine orange viability assay. Significant melanocyte cytotoxicity was seen in sera from patients with both active and inactive vitiligo(p<0.01). Melanocyte cytotoxicity measured with complement-mediated cytotoxicity decreased after systemic steroid treatment(p<0.05) ; however melanocyte cytotoxicity showed no significant change with systemic PUVA therapy.
Acridine Orange ; Antibodies ; Autoantibodies* ; Complement Activation ; Ethidium ; Humans ; Melanocytes ; PUVA Therapy ; Vitiligo*