Evaluation of Various Polishing Systems for Lithium Disilicate Glass-Ceramics.

OBJECTIVE: The aim of this study was to investigate the surface roughness of lithium disilicates (LS2s) polished using various polishing systems. MATERIALS AND METHODS: Two types of LS2 (A, Amber Mill and E, IPS e.max CAD) were polished using LS2-specific polishing systems (L-Edenta, L-Jota), a zirconia-specific polishing system (Z-Jota), and a conventional ceramic polishing system (P-Shofu) (n = 8 per group). The compositions of different polishing systems were analyzed using EDS. Surface roughness was measured using confocal laser scanning microscopy and analyzed using EDS and SEM. ANOVA and Tukey's tests were used for the statistical analyses (p = 0.05). RESULTS: The polishing systems were mainly composed of C, O, and Si. The L-Jota group exhibited rougher surfaces than the other groups. Amber Mill exhibited higher surface roughness than IPS e.max CAD (p⟨0.001). Among the polishing systems, the L-Jota group presented the highest roughness value (pp⟨0.001). The surface roughness of the AL-Jota group was higher than that of the other groups. CONCLUSIONS: A sufficiently smooth surface can be achieved without a LS2-specific polishing system. Further, the same polishing system can have different effects depending on the type of LS2.

Preoperative MRI Evaluation of Thyroid Cartilage Invasion in Patients with Laryngohypopharyngeal Cancer: Comparison of Contrast-Enhanced 2D Spin-Echo and 3D T1-Weighted Radial Gradient Recalled-Echo Techniques.

BACKGROUND AND PURPOSE: Accurate assessment of thyroid cartilage invasion on preoperative imaging influences management in patients with laryngeal and hypopharyngeal cancers. We evaluated the clinical usefulness of contrast-enhanced 3D T1-weighted radial gradient recalled-echo for preoperative assessment of thyroid cartilage invasion in patients with laryngohypopharyngeal squamous cell carcinoma, compared with 2D spin-echo T1WI. MATERIALS AND METHODS: Preoperative MR images of 52 consecutive patients who were diagnosed with laryngeal or hypopharyngeal cancer and underwent partial or total laryngectomy were analyzed. Pathologic specimens served as reference standards. Two independent head and neck radiologists evaluated the presence of thyroid cartilage invasion in both contrast-enhanced 2D spin-echo T1WI and 3D gradient recalled-echo sequences. The sensitivity, specificity, and accuracy of the 2 modalities were compared. The area under the curve was a measure of diagnostic performance. RESULTS: Pathologic neoplastic thyroid cartilage invasion was identified in 24 (46.2%) of the 52 patients. The sensitivity (75.0%), specificity (96.4%), and accuracy (86.5%) of contrast-enhanced 3D gradient recalled-echo were significantly higher than those of 2D spin-echo T1WI (58.3%, 89.3%, and 75.0%; P = .017, .003, and .002, respectively). 3D gradient recalled-echo had significantly better diagnostic performance (area under the curve = 0.963) than 2D spin-echo T1WI (area under the curve = 0.862; P = .010). CONCLUSIONS: Contrast-enhanced 3D gradient recalled-echo was diagnostically superior in identifying neoplastic thyroid cartilage invasion compared with 2D spin-echo T1WI in patients with laryngohypopharyngeal cancer, and therefore, may provide more accurate preoperative staging.

Measurement of hydraulic pressure on the sinus membrane for safer control during transcrestal sinus lifting.

The aim of this study was to determine the hydraulic pressures necessary to separate and lift the sinus membrane from the sinus floor in order to ensure a more controlled and safer hydraulic transcrestal sinus lifting surgery and prevent sinus membrane perforation. A flow-regulating hydrodynamic device with a pressure sensor was used in nine patients. The hydraulic pressure was found to increase steadily up to a mean peak of 25.0±13.0kPa, which is comparable to the medium suction power of ordinary vacuum cleaners. Subsequently, there was a short plateau followed by a sharp decrease in the hydraulic pressure.

MRI Features May Predict Molecular Features of Glioblastoma in Isocitrate Dehydrogenase Wild-Type Lower-Grade Gliomas.

BACKGROUND AND PURPOSE: Isocitrate dehydrogenase (IDH) wild-type lower-grade gliomas (histologic grades II and III) with epidermal growth factor receptor (EGFR) amplification or telomerase reverse transcriptase (TERT) promoter mutation are reported to behave similar to glioblastoma. We aimed to evaluate whether MR imaging features could identify a subset of IDH wild-type lower-grade gliomas that carry molecular features of glioblastoma. MATERIALS AND METHODS: In this multi-institutional retrospective study, pathologically confirmed IDH wild-type lower-grade gliomas from 2 tertiary institutions and The Cancer Genome Atlas constituted the training set (institution 1 and The Cancer Genome Atlas, 64 patients) and the independent test set (institution 2, 57 patients). Preoperative MRIs were analyzed using the Visually AcceSAble Rembrandt Images and radiomics. The molecular glioblastoma status was determined on the basis of the presence of EGFR amplification and TERT promoter mutation. Molecular glioblastoma was present in 73.4% and 56.1% in the training and test sets, respectively. Models using clinical, Visually AcceSAble Rembrandt Images, and radiomic features were built to predict the molecular glioblastoma status in the training set; then they were validated in the test set. RESULTS: In the test set, a model using both Visually AcceSAble Rembrandt Images and radiomic features showed superior predictive performance (area under the curve = 0.854) than that with only clinical features or Visually AcceSAble Rembrandt Images (areas under the curve = 0.514 and 0.648, respectively; P < . 001, both). When both Visually AcceSAble Rembrandt Images and radiomics were added to clinical features, the predictive performance significantly increased (areas under the curve = 0.514 versus 0.863, P < .001). CONCLUSIONS: MR imaging features integrated with machine learning classifiers may predict a subset of IDH wild-type lower-grade gliomas that carry molecular features of glioblastoma.

The rivaroxaban-adjusted normalized ratio: use of the prothrombin time to monitor the therapeutic effect of rivaroxaban.

Background: The prothrombin time may be used to monitor the plasma concentration of rivaroxaban. However, there is variability in the responsiveness of rivaroxaban to different thromboplastins. We aimed to develop a rivaroxaban-monitoring method using the prothrombin time to reduce the differences in the sensitivity among reagents. Methods: Rivaroxaban-spiked pooled normal plasma at a 0-1000 ng/ml concentration was used to generate a rivaroxaban-adjusted sensitivity index (SI) values, and was tested with three thromboplastins. The warfarin-adjusted international sensitivity index (ISI-warfarin), rivaroxaban-adjusted sensitivity index (SI-rivaroxaban), international normalized ratio (INR) calculated with ISI-warfarin, normalized ratio (NR) calculated with SI-rivaroxaban, and their coefficient of variances (CVs) were compared. The NR-rivaroxaban value was compared with the results of an anti-Xa assay. Results: The ISI-warfarin and SI-rivaroxaban using different thromboplastins were 1.02 and 1.88, respectively, with Thromborel S, 0.90 and 1.00 using Recombiplastin 2G, and 1.30 and 1.15 using Neoplastin CI-plus. Between-thromboplastin variability expressed as CV were 6.3%-25.1% when expressed as INR-warfarin and 1.7%-4.7% when expressed as NR-rivaroxaban. CVs for the NR-rivaroxaban with another laboratory were significantly lower than those for INR-warfarin. Anti-Xa assay v NR-rivaroxaban correlation coefficients were 0.97-0.99. Conclusion: Using a rivaroxaban-specific NR effectively minimises inter-thromboplastin variability. By utilizing a NR-rivaroxaban, standardized prothrombin time results could be rapidly obtained, especially useful in standardizing the therapeutic effect of rivaroxaban.

The relationship between clinical characteristics including presence of exposed lesions and health-related quality of life (HRQoL) in patients with psoriasis: analysis from the nationwide epidemiologic study for psoriasis in Korea (EPI-PSODE study).

BACKGROUND: Psychological aspect and quality of life should be considered in treating patients with psoriasis. OBJECTIVE: We sought to ascertain which clinical characteristics including presence of exposed lesions are associated with impairment of health-related quality of life (HRQoL) in patients with psoriasis. METHODS: The EPI-PSODE study was a nationwide, multicenter, cross-sectional study conducted in Korea that included 1260 adult patients with psoriasis. In addition to clinical characteristics including presence of exposed lesions, data were collected using the Psoriatic Arthritis (PsA) Screening and Evaluation (PASE), Dermatology Life Quality Index (DLQI), MOS 36-Item Short-Form Health Survey (SF-36), Work Productivity and Activity Impairment Questionnaire Psoriasis (WPAI: PSO) and Medication Satisfaction Questionnaire (MSQ). RESULTS: Patients with a DLQI score > 5 (n = 990) were younger, had an earlier onset of psoriasis, scored higher on the Psoriasis Area and Severity Index (PASI), had higher body surface area (BSA) and had higher PASE scores than patients with DLQI ≤ 5 (n = 266). The group of patients with exposed lesions (n = 871) were younger and male predominance, earlier onset of psoriasis, longer disease duration, higher PASI/BSA score and a higher proportion with drinking and smoking history each than the group of patients without exposed lesions (n = 389). Presence of exposed lesions negatively influenced DLQI, 36-Item Short-Form Health Survey (SF-36) (mental component), presenteeism, total work productivity impairment and total activity impairment in the WPAI: PSO. In multiple regression model, PASI score was the only variable which was significantly associated with all HRQoL measures. Presence of exposed lesions was a significant factor affecting DLQI and SF-36 (mental). CONCLUSION: The presence of exposed lesions has a negative impact on quality of life, mental health and work productivity. Therefore, effective treatments are particularly needed for psoriasis patients with exposed lesions.

Validation of reflex testing rules and establishment of a new workflow for body fluid cell analysis using a Sysmex XN-550 automatic hematology analyzer.

INTRODUCTION: We developed and validated reflex testing rules for the microscopic examination (ME) of body fluids (BFs) on the Sysmex XN-550 (Sysmex Corporation) instrument. METHODS: We assessed the detection limits, precision, linearity, and carryover. To develop the reflex testing rules (derivation arm), we tested 515 samples and then validated the rules using another 507 samples (validation arm). RESULTS: All analytical performances were acceptable, and the carryover was negligible. There was agreement between the automated count and ME of red blood cells (r = .98) and total nucleated cells (TNCs) (r = .98), as well as the differential counts of neutrophils (r = .90) and lymphocytes (r = .84). We developed reflex testing rules: TNCs <10/µL, cell 2/cell 1 ratio ≤0.7, HF-BF cells >7.9/100 white blood cells, LY-X ≥85 or LY-Y ≥90, and eosinophils >2.5%. In the validation arm, implementation of the rules resulted in 126 rule-negative samples (24.9%) that were well correlated between the 2 methods. We propose a new workflow for BF cell analysis based on automated counting. CONCLUSION: The Sysmex XN-550 can be a suitable alternative to ME for BF cell analysis, especially for screening samples and subsequent automatic reporting under the rational use of laboratory-specific rules.

Factors affecting quality of life in patients with vitiligo: a nationwide study.

BACKGROUND: Little is known about factors affecting the quality of life (QoL) of patients with vitiligo, and previous studies have shown conflicting results. OBJECTIVES: To explore the QoL of patients with vitiligo and to identify factors affecting QoL. METHODS: A nationwide questionnaire-based study was conducted with 1123 patients with vitiligo recruited from 21 hospitals in Korea from July 2015 to June 2016. Data were collected using a structured questionnaire for demographic information and the Skindex-29 instrument. Mild or severely impaired QoL in patients with vitiligo was assessed according to each domain (symptoms, functioning and emotions) of Skindex-29. Multivariate logistic regression analyses were performed to determine the factors associated with QoL. RESULTS: Of the enrolled participants, 609 were male and 514 female, with a mean age of 49·8 years (range 20-84). The median duration of disease was 3·0 years (range 0-60). Using multivariate logistic regression modelling, the involvement of visible body parts and a larger affected body surface area were consistently associated with QoL impairment in all three domains of Skindex-29. Additionally, the QoL of patients aged 20-59 years, who potentially had a more active social life than older patients, was associated with functional impairment. Furthermore, a higher educational background was associated with emotional impairment. CONCLUSIONS: A multitude of factors significantly influence the QoL of patients with vitiligo. A better appreciation of these factors would help the management of these patients.

Full-genome sequence analysis of an uncommon norovirus genotype, GII.21, from South Korea.

Noroviruses (NoVs) are major causal agents of acute gastroenteritis in humans. NoV GII.4 is the predominant genotype globally. However, uncommon and minor types of NoVs are consistently detected and some have been shown to dominate over GII.4. Therefore, the prevalence of dominant and uncommon NoVs makes the identification of these viruses important for the prediction and prevention of pandemics. In this study, the full-genome sequence of a NoV (strain JW) detected in Korea was extensively characterized. The full-length genome was 7510 nucleotides long, and phylogenetic analysis based on the whole-genome sequences, including open reading frame (ORF)1, ORF2, and ORF3, indicated that it belonged to the GII.21 genotype. Strain JW showed maximum identity with strain YO284; however, comparison of the amino acid sequence of ORF2, which functions as an antigen, showed substitutions in several amino acids. GII.21 is not a prevalent epidemiological agent of acute gastroenteritis in humans, but it is consistently found in gastroenteritis patients from several countries. The present study provides the first full-genome sequence analysis of NoV GII.21 isolated from a patient in Korea. Our findings provide not only valuable genome information but also data for epidemiology studies, epidemic prevention, and vaccine development strategies.

Optimal maintenance treatment with calcipotriol/betamethasone dipropionate gel in Korean patients with psoriasis vulgaris: a multicentre randomized, controlled clinical trial.

BACKGROUND: There is a lack of response data for topical treatments for psoriasis vulgaris in Asian patients. OBJECTIVES: To determine the optimal maintenance regimen for topical treatment with calcipotriol monohydrate/betamethasone dipropionate gel in Korean patients with psoriasis vulgaris, by comparing the efficacy of three 8-week maintenance regimens. METHODS: This was a multicentre, prospective, randomized, controlled, parallel-group, open-label, phase 4 clinical trial, conducted in South Korea. Patients with psoriasis vulgaris on the limbs/trunk received once-daily treatment with calcipotriol monohydrate (50 µg/g)/betamethasone dipropionate (500 µg/g) gel for 8 weeks (induction phase). Responders (defined as an Investigator's Global Assessment of Disease Severity (IGA) grade of 'clear' or 'almost clear') were then randomized to receive 8 weeks' maintenance treatment with Xamiol® gel once daily as needed [pro re nata (PRN Group)], once daily every day (Continuous group), or twice weekly - on Saturday and Sunday (Weekend group). The primary endpoint was the percentage of IGA responders at week 16. RESULTS: At the end of the induction phase, 62.18% of patients were IGA responders. At the end of the maintenance phase (week 16), the responder rate was 63.89% for the PRN group, 67.5% for the Continuous group and 31.43% for the Weekend group. The PRN and Continuous groups were statistically superior to the Weekend group (P = 0.0109 and P = 0.0015), but the PRN and Continuous groups did not differ statistically. The incidence of adverse events did not differ significantly between the groups. CONCLUSION: Among Korean patients with psoriasis vulgaris, maintenance treatment with calcipotriol monohydrate/betamethasone dipropionate using a continuous daily regimen or an 'as needed' daily regimen provided similar efficacy, whereas a twice-weekly regimen was significantly less efficacious than either of these regimens.

The added values of multiplex reverse transcriptase-PCR followed by mutation screening in the initial evaluation of acute leukemia.

INTRODUCTION: This study investigates the benefits of using multiplex reverse transcriptase-PCR (RT-PCR) in addition to standard karyotyping during the initial evaluation of acute leukemia. METHODS: A total of 1114 consecutive specimens from patients with acute leukemia were tested using a commercial multiplex RT-PCR kit (HemaVision, DNA Diagnostic). NPM1 and CEBPA mutations were selectively tested in acute myeloid leukemia (AML) patients with multiplex RT-PCR negativity. RESULTS: In specimens with optimal cytogenetics, the frequency of recurrent translocations was 31.3%, and cryptic translocations were detected in 2.1% of samples. The concordance rate between karyotyping and multiplex RT-PCR was 97.5%. In addition to the established functions, we demonstrated the additional benefits of multiplex RT-PCR, including successful molecular characterization, even in cytogenetically suboptimal specimens (5.7%); detection of submicroscopic aberrations (1.0%); detection of rare but potentially significant translocations or variants (2.5%); selection of AML candidates for mutation analysis (68.3%); and finally exclusion of recurrent translocations in patients with acute lymphoblastic leukemia or mixed phenotype acute leukemia (22.5%). CONCLUSION: We reconfirmed the accuracy and reliability of multiplex RT-PCR for diagnosing acute leukemia and demonstrated additional advantages of this system for the initial evaluation of acute leukemia. Thus, multiplex RT-PCR is worth considering in diagnostic testing of acute leukemias.

Involvement of T cells in early evolving segmental vitiligo.

Recent studies have suggested an overlapping autoimmune mechanism between segmental vitiligo (SV) and nonsegmental vitiligo (NSV). Although T-cell infiltration is observed in the margins of active lesions in NSV, the histopathological characteristics of the active margin of SV are not well known. To determine if T-cell inflammatory responses are present in the active margin of SV lesions, biopsies were taken from the active margin of a lesion in 12 patients with early or actively spreading SV and compared with a normal control sample (on the symmetrical, opposite site of the same dermatome). The samples were stained for CD4, CD8, CD25 and interferon-γ. Lymphocytic infiltration was seen in 70% of patients. CD4+ T cells infiltrated the dermis, while CD8+ T cells were present in the epidermis or attached to the basal layer. The increase in the number of CD8+ T cells was significant (P < 0.04), while CD4+ or CD25+ T cells also appeared to be increased in number, but this was not significant. These results suggest that SV also has an autoimmune mechanism in the early evolving stage.

Haploidentical HCT using an αß T-cell-depleted graft with targeted αß(+) cells by add-back after a reduced intensity preparative regimen containing low-dose TBI.

Between 2012 and 2015, 42 pediatric patients underwent haploidentical hematopoietic cell transplantation using an αß(+) T-cell-depleted graft with targeted αß cells at 1-5 × 10(5)/kg by add-back; 31 had hematologic malignancy (HM), 8 had non-malignant disease (NM) and 3 had solid tumors. All patients received uniform reduced-intensity conditioning with fludarabine, cyclophosphamide, rabbit anti-thymocyte globulin and low-dose TBI. All 42 patients achieved neutrophil engraftment at a median of 10 days. The cumulative incidences (CIs) of ⩾grade II and ⩾grade III acute GvHD were 31±7.1% (SE) and 12±5.0%, respectively, and 1-year CI of chronic GvHD was 15±5.8%. One patient died of CMV pneumonia, leading to transplant-related mortality (TRM) of 2.6±2.5%. Sixteen patients relapsed and 11 died of disease. At a median follow-up of 19 months (range, 5-43 months), the estimated 2-year event-free survival for NM and HM were 88±11.7 and 50±10.1%, respectively. Our study demonstrated that haploidentical hematopoietic cell transplantation after ex vivo depletion of αß(+) T cells with targeted dose noticeably reduced the graft failure rate and TRM in pediatric patients and could be applied to patients lacking a suitable related or unrelated donor.

Evaluation of parameters obtained from the Sysmex XN-2000 for predicting the recovery of the absolute neutrophil count and platelets after hematopoietic stem cell transplantation.

INTRODUCTION: We analyzed abilities of parameters from Sysmex XN-2000 (Sysmex, Kobe, Japan) to predict absolute neutrophil count (ANC) and platelet recovery after hematopoietic stem cell transplantation (HSCT) in patients with hematologic malignancies. METHODS: We prospectively analyzed 911 follow-up peripheral blood samples from 44 HSCT-performed patients and evaluated the performances of the following parameters: WBC, immature granulocyte (IG), hematopoietic stem and progenitor cells (HPC), immature reticulocyte fraction (IRF), immature platelet fraction (IPF), platelet distribution width (PDW), mean platelet volume (MPV), and platelet larger cell ratio (P-LCR). RESULTS: When compared to four other parameters, the identification of initiation in IG (%)/HPC (%) increase enabled earlier prediction of ANC recovery to >500/µL and >1000/µL with more time benefit of 3.5-6.5 days/2.0-5.0 days and 3.0-6.0 days/2.0-5.0 days, respectively. When compared to IPF (%), the identification of initiation in PDW, MPV, and P-LCR (%) increase enabled earlier prediction of platelet recovery to >20 000/µL and >50 000/µL with more time benefit of 2.5-3.5 days and 2.0-3.0 days, respectively. However, the standard deviation of time benefit obtained from IG (%)/HPC (%)/PDW/MPV/P-LCR (%) was consistently large (3.0-4.3 days). CONCLUSIONS: There is a systematic pattern where a rise in most of the studied parameters can be observed in most patients before ANC/platelet recovery. However, the interindividual variation between the time of rise of these parameters and ANC/platelet recovery is large, and therefore, using these parameters to predict recovery in the individual patient is probably not meaningful in the clinical setting.

Integrin α11ß1 regulates cancer stromal stiffness and promotes tumorigenicity and metastasis in non-small cell lung cancer.

Integrin α11ß1 is a stromal cell-specific receptor for fibrillar collagens and is overexpressed in carcinoma-associated fibroblasts (CAFs). We have investigated its direct role in cancer progression by generating severe combined immune deficient (SCID) mice deficient in integrin α11 (α11) expression. The growth of A549 lung adenocarcinoma cells and two patient-derived non-small cell lung carcinoma (NSCLC) xenografts in these α11 knockout (α11(-/-)) mice was significantly impeded, as compared with wild-type (α11(+/+)) SCID mice. Orthotopic implantation of a spontaneously metastatic NCI-H460SM cell line into the lungs of α11(-/-) and α11(+/+) mice showed significant reduction in the metastatic potential of these cells in the α11(-/-) mice. We identified that collagen cross-linking is associated with stromal α11 expression, and the loss of tumor stromal α11 expression was correlated with decreased collagen reorganization and stiffness. This study shows the role of integrin α11ß1, a receptor for fibrillar collagen in differentiation of fibroblasts into CAFs. Furthermore, our data support an important role for α11 signaling pathway in CAFs, promoting tumor growth and metastatic potential of NSCLC cells and being closely associated with collagen cross-linking and the organization and stiffness of fibrillar collagen matrices.

Body fluid cellular analysis using the Sysmex XN-2000 automatic hematology analyzer: focusing on malignant samples.

INTRODUCTION: The majority of previous studies on body fluid (BF) mode of automatic hematology analyzer used nonmalignant BF samples. Here, we evaluated the BF mode on the recently launched Sysmex XN for counting blood cells, especially for malignant samples. METHODS: A total of 405 BF specimens including 125 malignant samples were analyzed using both the automated method and manual microscopy. RESULTS: In non-cerebrospinal fluids (CSF) samples, there was an agreement between two methods for WBC, RBC, polymorphonuclear, and mononuclear cell counts (R(2)  = 0.96, 0.94, 0.88, and 0.88, respectively). CSF samples showed slightly poorer correlations than other fluids. Exclusion of malignant samples significantly improved correlations in non-CSF samples, but not in CSF samples. High fluorescence-BF (HF-BF) cells were identified significantly more frequently in malignant samples compared to benign samples (17.8 and 4.15/100 WBC, respectively; P < 0.001). Receiver operating characteristic curve analysis demonstrated an HF-BF cell AUC of 0.791 using a cutoff value of 6.9/100 WBC for detecting malignant samples. CONCLUSION: The BF mode on the Sysmex XN could be an alternative method for the manual counts in the BF analysis with a few drawbacks. However, if a concentration of HF-BF cells is greater than the given threshold, microscopic examination should be subsequently performed.

Sepsis affects most routine and cell population data (CPD) obtained using the Sysmex XN-2000 blood cell analyzer: neutrophil-related CPD NE-SFL and NE-WY provide useful information for detecting sepsis.

INTRODUCTION: The Sysmex XN-2000 analyzer can assess 36 routine and 57 cell population data (CPD) items. In this study, we evaluated these items as sepsis biomarkers. METHODS: We enrolled 280 normal control (NC) and 130 sepsis patients. The sepsis patients were classified as uncomplicated or complicated sepsis. Routine and CPD items were determined, and the results were compared at between the NC and sepsis groups, uncomplicated and complicated sepsis groups, and survivors and nonsurvivors. RESULTS: For the detection of sepsis, CPD items NE-SFL [defined as the fluorescent light intensity of the neutrophil area on the WDF (white blood cell differential) scattergram] and NE-WY (defined as the fluorescent light distribution width of the neutrophil area on the WDF scattergram) showed comparative or higher AUC of 0.909 and 0.905, respectively, when compared with routine items such as hematocrit, hemoglobin, RBC, RDW, immature granulocytes count, lymphocytes count, and neutrophils count. For the discrimination of sepsis severity, only platelet-related items showed higher AUC (0.723 - 0.748) than lactic acid (0.695). For the prediction of 28-day mortality, only CV and SD of RDW showed higher AUC (0.766 and 0.732 each) than lactic acid (0.712). CONCLUSIONS: Sepsis patients demonstrated significant changes in routine and CPD items related to RBC, neutrophils, lymphocytes, and platelets when compared to NCs. Increase in CPD items NE-SFL and NE-WY, which may indicate neutrophil immaturity or activation, could be useful for the detection of sepsis patients, in conjunction with currently used surrogate sepsis biomarkers. However, these items did not efficiently contribute to the discrimination of sepsis severity or predict mortality.

Refinement of treatment strategies in ex vivo T-cell-depleted haploidentical SCT for pediatric patients.

We evaluated the feasibility of T-cell-depleted haploidentical hematopoietic SCT (HHCT) in pediatric patients. Between July 2008 and January 2013, 28 patients underwent ex vivo T-cell-depleted HHCT; 9 had hematologic malignancy, 18 had nonmalignant hematologic disease, and 1 had refractory neuroblastoma. Twenty-six patients achieved neutrophil engraftment at a median of 11 days (range, 9-15 days). Two patients failed to achieve primary engraftment and five experienced graft rejection after primary engraftment. These seven patients achieved stable engraftment after a second HHCT. The cumulative incidences (CIs) of⩾grade II and⩾grade III acute GVHD were 33.3% and 14.3%, respectively, and the 1-year CI of extensive chronic GVHD was 11.1%. Four patients died of non-relapse-related causes (two of CMV disease, one of encephalopathy and one of autoimmune hemolytic anemia) and one of leukemia relapse. Non-relapse mortality at 100 days, 1 year and 2 years was 0.0%, 10.7% and 14.3%, respectively. At a median follow-up of 32.8 months (range, 17.0-72.5 months), the 2-year OS was 82.1%. OSs for nonmalignant diseases and malignant diseases were 94.4% and 60.0%, respectively (P=0.019). Thus, HHCT is a realistic alternative for patients with malignant or nonmalignant diseases who lack a suitable donor.

FISH analysis of MLL gene rearrangements: detection of the concurrent loss or gain of the 3' signal and its prognostic significance.

INTRODUCTION: The rearrangement of the mixed-lineage leukemia (MLL) gene occurs through translocations and insertions involving a variety of partner chromosome genes. However, there are few studies on aberrant MLL signal patterns such as concurrent 3' MLL deletion. METHODS: A total of 84 patients with acute leukemia (AL) who had MLL rearrangements detected by florescence in situ hybridization (FISH) were enrolled in the study. The distribution of MLL fusion partner genes was analyzed, and aberrant MLL signals were evaluated. RESULTS: Seventy-seven (91.7%) patients had MLL rearrangements, involving previously described translocation partner genes (TPGs). Among these TPGs, the frequencies of MLLT3, AFF1, MLLT4, and ELL were 29.8%, 17.9%, 15.5%, and 13.1%, respectively. A high frequency of MLLT4 in our study was due to the high proportion of acute myeloid leukemia cases in pediatric and adult patients. Aberrant MLL signals were found in 18 patients: 11 (61.1%) with 3' MLL signal loss and 7 with 3' MLL signal gain. All cases with 3' MLL signal gain were due to an extra derivative partner chromosome. The median overall survival period of patients with 3' MLL gain was shorter than that in patients without aberrant MLL signal patterns. CONCLUSION: Aberrant MLL signals were frequently detected by FISH analysis. The 3' MLL gain was associated with poor prognosis in patients with AL. Therefore, it is important to detect aberrant MLL signal patterns using FISH analysis.

Morphologic detection of blast cells in the cerebrospinal fluid at diagnosis of adult acute lymphoblastic leukemia appears to be associated with adverse prognosis.

INTRODUCTION: Appropriate treatment of central nervous system (CNS) involvement in adult acute lymphoblastic leukemia (ALL) is important for patient prognosis, but the diagnostic criteria of CNS involvement has not been established. METHODS: The significance of blast cells in the cerebrospinal fluid (CSF) at diagnosis was evaluated in 81 adults newly diagnosed with ALL. Patients with unequivocal morphologic evidence of lymphoblasts in the cytocentrifuged CSF slide were considered to have CNS involvement regardless of the leukocyte count. The outcomes of the patients were analyzed. RESULTS: Four of the 81 patients (5%) had detectable blast cells, and three of these four patients had less than five leukocytes/µL of CSF. One-year event-free survival (EFS) was 25.0% and 53.2% (P = 0.008) and overall survival (OS) was 50.0% and 68.8% (P = 0.001) in patients with and without CNS involvement, respectively. CNS involvement had prognostic impact on EFS (P = 0.047) and OS (P = 0.009) after adjusting for sex, age, leukocyte count, Philadelphia chromosome status, and immunophenotype. CONCLUSION: This study suggests that morphologic detection of blast cells in the CSF at diagnosis of adult ALL, regardless of the leukocyte count, is associated with adverse prognosis.