We report a case of dural-based intracranial primary mesenchymal chondrosarcoma, initially thought to be a meningioma. This rare tumor should be included in the differential diagnosis of an aggresive dural-based lesion occurring in a young adult. A 27-year-old man presented with headache, nausea and vomiting, first experienced months earlier. Pre-enhanced CT revealed the presence of a well-marginated isodense mass with dense calcifications in the frontoparietal convexity, while MR images depicted a lobulated extra-axial mass with peritumoral edema. At T1-weighted imaging, the signal intensity of the mass was slightly low or than that of gray matter, while T2-weighted imaging demonstrated heterogeneous high signal intensity. Some portions of the tumor showed low signal intesnity at all sequences, suggesting the presence of calcification. After the injection of contrast medium, heterogeneous enhancement was observed. We report the radiologic findings of an intracranial primary mesenchymal chondrosarcoma, confirmed pathologically.
Adult ; Chondrosarcoma ; Chondrosarcoma, Mesenchymal* ; Diagnosis, Differential ; Edema ; Headache ; Humans ; Meningioma ; Nausea ; Vomiting ; Young Adult

Acute lymphoblastic leukemia (ALL), in general, can be diagnosed by detecting blasts in peripheral blood or bone marrow. Some of the cases of ALL do not show typical leukemic features, and only manifest as refractory anemia, thrombocytopenia, myelofibrosis and lymphocytic infiltration into bone marrow. Several months after presentation, they may reveal typical leukemic features and are diagnosed as ALL. This kind of leukemia is called ALL with aleukemic prodrome. Although the incidence of ALL with aleukemic prodrome is 1.5~2.2% of childhood ALL cases, it is rarely reported in Korea. We experienced a 6 month-old female infant who presented with refactory anemia and thrombocytopenia, and two serial of bone marrow examination revealed only myelofibrosis. She subsequently developed ALL 3 months later. We report this case with a brief review of related literatures.
Anemia ; Anemia, Refractory ; Bone Marrow ; Bone Marrow Examination ; Female ; Humans ; Incidence ; Infant ; Korea ; Leukemia ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Primary Myelofibrosis ; Thrombocytopenia

BACKGROUND: The majority of patients undergoing stem cell transplantation (SCT) require a blood transfusion until the complete engraftment. Because blood transfusion rules for patients with ABO-incompatible SCT are complicated, we developed an ABO-incompatible transfusion management system (ABO-ITMS) for accurate blood transfusion and improved manageability. METHODS: A committee composed of medical doctors, technicians, and a programmer developed ABO-ITMS during the eight months from July 2013 to February 2014. The program has been linked with other databases, including clinical and laboratory databases and resulted in a new subsystem of the health information system. Server computer's operating system was Window Server 2008, and the database manager program was Oracle 11g. Programming language was ASP.Net (VBScript, C #), and the server and client computer were used to connect to the web server using a web browser. RESULTS: ABO-ITMS was designed to follow three main steps by hematologic oncology clinic, laboratory physician, and blood bank. In the first step, a hematologic-oncology clinic doctor inputs SCT recipients' data and appropriate ABO group for each phase of post-transplantation. Laboratory physician enters the isoagglutinin titer and ABO group at the second step. Finally, blood bank workers enter the results of type, screening, and antibody identification. The patient's SCT information and the previous immunohematologic test results are shown on the screen. CONCLUSION: ABO-ITMS can replace the existing complicated system and workflow. ABO-ITMS will contribute to reducing medical error and improving quality of SCT recipient care.
Blood Banks ; Blood Transfusion* ; Health Information Systems ; Humans ; Mass Screening ; Medical Errors ; Programming Languages ; Stem Cell Transplantation* ; Web Browser

BACKGROUND: Although heart rate variability (HRV) and blood pressure variability (BPV) arise from many different influences, probably the most consistent external modulator is respiration. At rest, the heart rate increases on inspiration and decreases on expiration, a phenomenon called respiratory sinus arrhythmia (RSA). Spectral analysis of heart rate offers good and reproducible estimate of RSA and BPV. Many studies have been conducted on the effects of respiration on HRV and BPV during awake subject breathing spontaneously. However, little is known as to whether respiratory rate modulates HRV and BPV during general anesthesia with mechanical ventilation. Here, we studied effects of respiratory rate on HRV and BPV during general anesthesia. METHODS: We studied 40 patients undergoing general anesthesia with mechanical ventilation. Maintaining anesthesia with isoflurane, we recorded R-R interval and systolic blood pressure at respiratory rate of 15, 10 and 6 breaths/minute. Data was analyzed by the power spectral analyses of HRV and BPV, which were divided into low frequency (LF) and high frequency (HF) band. RESULTS: Respiratory rate did not affect RR interval, systolic blood pressure, and total spectral power HRV and BPV. Compared with its value at 15 breaths/minute, HF-HRV was significantly increased at 6 breaths/minute. HF-and LF-BPV at 6 breaths/minute were significantly increased versus 15 breaths/minute. CONCLUSIONS: Respiratory rate modulates HRV and BPV during general anesthesia with mechanical ventilation. We suggest that respiratory rate should be considered and controlled in studies of cardiovascular variability during general anesthesia.
Anesthesia ; Anesthesia, General* ; Arrhythmia, Sinus ; Blood Pressure ; Heart Rate ; Humans ; Isoflurane ; Respiration ; Respiration, Artificial* ; Respiratory Rate*

In the past decade, clinical microbiology underwent revolutionary changes in methods used to identify microorganisms, a transition from slow and traditional microbial identification algorithms to rapid molecular methods and mass spectrometry (MS). Earlier, MS was clinically used as a highly complex method that was adapted for protein-centered analysis of samples in chemistry laboratories. Recently, a paradigm-shift happened when matrix-assisted laser desorption ionization-time of flight (MALDI-TOF) MS was implemented to be used in microbiology laboratories for rapid and robust methods for accurate microbial identification. Two instrument platforms, marketed by well-established manufacturers, are beginning to displace automated phenotypic identification instruments and in some cases even genetic sequence-based identification practices. This review summarizes the current role of MALDI-TOF MS in clinical research, in diagnostic clinical microbiology laboratories, and serves as an introduction to MALDI-TOF MS, highlighting research associated with sample preparation, algorithms, interpretations, and limitations. Currently available MALDI-TOF MS instruments as well as software platforms that support the use of MALDI-TOF with direct specimens have been discussed in this review. Finally, clinical laboratories are consistently striving to extend the potential of these new methods, often in partnership with developmental scientists, resulting in novel technologies, such as MALDI-TOF MS, which could shape and define the diagnostic landscape for years to come.
Chemistry ; Mass Spectrometry

BACKGROUND AND OBJECTIVES: Left ventricle burdened by longstanding volume-overload, undergoes various structural and functional alterations. Accordingly, the expressions of multiple classes of genes are likely to be altered. However, the profile of gene expressions, specifically in a volume-overloaded left ventricle in humans, has not been explored. SUBJECTS AND METHODS: The pattern of gene expression was studied, using a cDNA microarray, in myocardium from 4 normal subjects and 5 patients with chronic regurgitant valvular heart disease whose end-diastolic left ventricular dimension measures 65 mm or more, but whose systolic function remained preserved. RESULTS: We identified 58 differentially expressed genes that were functionally classifiable in the volume-overloaded myocardium. Those genes involved in cell cycle/growth (up/down-regulation: 9/1), signal transduction (4/1) were mostly overexpressed in the volume-overloaded myocardium. The distributions of the gene expressions were variable for those involved in transcription/translation (up/down-regulation: 6/7) and apoptosis (2/2). The genes related to the myocyte structure (troponin T3, tropomyosin, etc)(up/down-regulation: 1/10), as well as those related to metabolism (2/5), were underexpressed. The gene expression patterns from RT-PCR and Western blot, with randomly selected genes, were similar to those from the cDNA microarray. CONCLUSION: Altered expression was identified in multiple genes in the volume-overloaded human left ventricle prior to the development of heart failure. The genes related to cell growth and signal transduction were mostly overexpressed, while those related to cellular structure and metabolism appeared to be underexpressed. These results might help in the elucidation of cellular mechanisms for the remodeling process associated with chronic volume-overloading.
Apoptosis ; Blotting, Western ; Cellular Structures ; Gene Expression* ; Heart Failure* ; Heart Valve Diseases ; Heart Ventricles ; Heart* ; Humans* ; Metabolism ; Muscle Cells ; Myocardium* ; Oligonucleotide Array Sequence Analysis ; Signal Transduction ; Transcriptome* ; Tropomyosin

BACKGROUND: The Rh blood group includes several antigens, of which D, C, E, c, and e are clinically important. Although nucleic acids from whole blood can be used for Rh blood group genotyping, it is also possible to genotype free circulating fetal nucleic acids from plasma and serum. We performed Rh blood group phenotyping and genotyping using nucleic acids from whole blood and free circulating nucleic acids from plasma and serum, respectively. The results were compared. METHODS: Forty-four blood samples were phenotyped and genotyped for RhD and RhCE blood groups. Phenotyping was performed by hemagglutination assay. Further tests were performed on RhD-negative samples. Nucleic acids were extracted from whole blood, plasma, and serum. Plasma and serum were prepared after filtration and genotyped by real-time polymerase chain reaction. RESULTS: RhD blood group results showed one (2.3%) discrepant case in which the DEL phenotype appeared wild RHD genotype. Among nucleic acids, there were seven discrepant results: two from plasma and five from serum based on whole blood nucleic acids. RhCE blood group results showed three (6.8%) phenotype-genotype discordances. Among nucleic acids, seven (15.9%mpared to phenotypes. Kappa coefficients of serum were lower than those of plasma. CONCLUSION: RHD and RHCE genotype could be identified by assaying free circulating nucleic acids in plasma or serum. This study suggests that plasma is more reliable than serum as a specimen for RHD and RHCE genotyping of free circulating nucleic acids.
Blood Group Antigens ; Filtration ; Genotype ; Hemagglutination ; Nucleic Acids* ; Phenotype ; Plasma* ; Real-Time Polymerase Chain Reaction

PURPOSE: Peripheral blood stem cell transplantation (PBSCT) has been widely used. The optimal time for collection is a critical factor to obtain proper counts of CD34 cell by peripheral blood stem cell collection (PBSC). The purpose of this study was to identify the factors influencing peripheral blood stem cell collection in order to figure out the more effective timing for PBSC. METHOD: The subjects of this study were 189 patients undergoing 3 leukapheresis from January 28, 2005 to December 31, 2006. Group's characteristics, checkup opinion of pre-peripheral blood on the day of harvest & outcome of PBSC were analyzed and evaluated using SAS statistics program after grouping patients as below; group 1-CD34 cell counts <2 x10(6)/kg (n=97); group 2-2x10(6)/kg < or =CD34 cell counts <4x10(6)/kg (n=26); group 3-CD34 cell counts > or =4x10(6)/kg (n=63). RESULTS: Based on outcome of peripheral blood stem cell according to diagnosis, acute myelocytic leukemia (AML) was 65.5% at Group 1, Lymphoma was 21.7% at Group 2 and multiple myeloma (MM) was 70.8% at Group 3. There were significant differences in CD34 cell counts according to diagnosis (p=0.00004). Type of cytokine mobilization according to diagnosis, Lenograsim was using 62.5% of MM & 38.2% of AML and filgrastim is using 22.0% of AML only. Circular peripheral blood CD34 cell counts prior to harvest was 258.1/microliter at Group 3 which was much higher comparing to Group 1 (10.5/microliter) and Group 2 (39.9/microliter) (p<0.001). TNC counts of collected peripheral blood stem cell was 15.36x10(6)/kg at Group 3 microliter and it's much higher than Group 2 (13.16x10(6)/kg) and Group 1 (12.36x10(6)/kg) (p=0.083). There was no significant difference in MNC counts inbetween 3 groups. CONCLUSIONS: Circular peripheral blood CD34+ cell counts prior to harvest was much higher at Group 3 than Group 1 and Group 2. Therefore, the number of CD34+ cells on the day of harvest can be used as an accurate predictor for peripheral blood stem cell.
Cell Count ; Granulocyte Colony-Stimulating Factor ; Humans ; Leukapheresis ; Leukemia, Myeloid, Acute ; Lymphoma ; Multiple Myeloma ; Peripheral Blood Stem Cell Transplantation ; Phenothiazines ; Recombinant Proteins ; Stem Cells ; Filgrastim

Purpose: Tyrosine kinase inhibitors (TKI) targeting vascular endothelial growth factor receptor (VEGFR) signaling pathways have been used for metastatic clear cell renal cell carcinoma (mCCRCC), but resistance to the drug develops in most patients. We aimed to explore the underlying mechanism of the TKI resistance with regard to programmed death-ligand 1 (PD-L1) and to investigate signaling pathway associated with the resistant mechanism. Materials and Methods: To determine the mechanism of resistance, 10 mCCRCC patients from whom tumor tissues were harvested at both the pretreatment and the TKI-resistant post-treatment period were included as the discovery cohort, and their global gene expression profiles were compared. A TKI-resistant renal cancer cell line was established by long-term treatment with sunitinib. Results: Among differentially expressed genes in the discovery cohort, increased PD-L1 expression in post-treatment tissues was noted in four patients. Pathway analysis showed that PD-L1 expression was positively correlated with the mammalian target of rapamycin (mTOR) signaling pathway. The TKI-resistant renal cancer cells showed increased expression of PD-L1 and mTOR signaling proteins and demonstrated aggressive tumoral behaviour. Treatment with mTOR inhibitors down-regulated PD-L1 expression and suppressed aggressive tumoral behaviour, which was reversed with stimulation of the mTOR pathway. Conclusion These results showed that PD-L1 expression may be increased in a subset of VEGFR-TKI–resistant mCCRCC patients via the mTOR pathway.

10% of labeled tumor cells) of TNF receptor 1 (TNFR1), the protein product of TNFRSF1A gene, was correlated with sarcomatoid dedifferentiation and was an independent predictive factor of clinically unfavorable response and shorter survivals in separated TKI-treated ccRCC cohort.CONCLUSION: TNF-α signaling may play a role in TKI resistance, and TNFR1 expression may serve as a predictive biomarker for clinically unfavorable TKI responses in ccRCC.]]>
Biomarkers ; Carcinoma, Renal Cell ; Cohort Studies ; Dataset ; Drug Resistance ; Gene Expression ; Gene Expression Profiling ; Heterografts ; Humans ; Immunohistochemistry ; Protein-Tyrosine Kinases ; Receptors, Tumor Necrosis Factor ; Receptors, Tumor Necrosis Factor, Type I ; Tumor Necrosis Factor-alpha