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This study considers low‐ability workers with the difference in their specialization areas and investigates the consequence of automation. There are two findings. First, owing to automation, it is more difficult for displaced workers than for other workers to find a new job, because these displaced workers lose their suitable jobs. Consequently, the unemployment rate increases with an increase in the number of displaced workers. Second, unless an additional increase in unsuitable jobs is large under the introduction of new tasks, automation does not necessarily increase the unemployment rate because it is less difficult for workers to find a new job. [ABSTRACT FROM AUTHOR]

von Willebrand factor ristocetin cofactor (vWF activity) and platelet count (PLT) are negatively correlated in patients with polycythemia vera (PV) and essential thrombocythemia (ET). However, vWF activity does not always normalize upon controlling PLT in those patients. To address this issue, we investigated the correlation between vWF activity and PLT in PV and ET patients. The negative correlation between vWF activity and PLT was stronger in calreticulin mutation‐positive (CALR+) ET than in Janus kinase 2 mutation‐positive (JAK2+) PV or ET groups. When PLT were maintained at a certain level (<600 × 109/L), low vWF activity (<50%) was more frequently observed in JAK2+ PV patients than in JAK2+ ET (p =.013) or CALR+ ET (p =.013) groups, and in PV and ET patients with ≥50% JAK2+ allele burden than in those with allele burden <50% (p =.015). High vWF activity (>150%) was more frequent in the JAK2+ ET group than in the CALR+ ET group (p =.005), and often associated with vasomotor symptoms (p =.002). This study suggests that some patients with JAK2+ PV or ET have vWF activity outside the standard range even with well‐controlled PLT, and that the measurement of vWF activity is useful for assessing the risk of thrombosis and hemorrhage. [ABSTRACT FROM AUTHOR]

Paragonimiasis is a parasitic disease caused by Paragonimus westermani infection, and migration to the brain results in cerebral paragonimiasis. Cerebral paragonimiasis is now extremely rare, but a few cases are still reported. A 48‐year‐old Japanese woman presented with right‐hand convulsion, right‐hand numbness, sputum, and fatigue. Chest computed tomography demonstrated multiple nodular lesions, and head computed tomography revealed a hemorrhagic lesion in the left motor cortex. Magnetic resonance imaging revealed multiple small ring‐shaped lesions with surrounding edema. Laboratory evaluation demonstrated peripheral eosinophilia. We considered eosinophilic granulomatosis with polyangiitis and started steroid treatment as a diagnostic therapy since we wanted to avoid cerebral lesion biopsy if possible. However, the patient underwent craniotomy surgery after steroid treatment for four months because a new intracerebral mass lesion had appeared. Trematode eggs were detected in the sample, and the final diagnosis was cerebral paragonimiasis. The patient was successfully treated with praziquantel. Cerebral paragonimiasis is extremely rare but should be considered in the differential diagnosis if atypical intracranial hemorrhage and peripheral eosinophilia are observed. [ABSTRACT FROM AUTHOR]

Keywords: consolidation chemotherapy; hematopoietic stem cell transplantation; peripheral T-cell lymphoma; pirarubicin; prognosis EN consolidation chemotherapy hematopoietic stem cell transplantation peripheral T-cell lymphoma pirarubicin prognosis 1094 1096 3 12/11/22 20221201 NES 221201 DATA AVAILABILITY STATEMENT The data that support the findings of this study are available on request from the corresponding author. The double-THP-COP protocol comprised three main phases: induction therapy with a single cycle of THP-COP, intensification therapy with three cycles of double-THP-COP, and consolidation therapies. Pirarubicin-based intensive chemotherapy followed by consolidative high-dose chemotherapies for peripheral T-cell lymphomas: A noncomparative phase 2 study. [Extracted from the article]

Aim: To evaluate subclinical left ventricular (LV) regional dysfunction in patients with primary Sjögren's syndrome (pSS) using feature tracking cardiac magnetic resonance (FT‐CMR) imaging and to identify pSS characteristics independently associated with LV regional dysfunction. Method: Fifty patients with pSS and 20 controls without cardiovascular disease underwent non‐contrast CMR imaging. Labial gland biopsy was performed in 42 patients (84%). Disease activity was assessed using the European League Against Rheumatism Sjögren's syndrome disease activity index (ESSDAI). LV global longitudinal strain (GLS), global circumferential strain (GCS), and global radial strain (GRS) were measured using FT‐CMR. Results: No significant differences in cardiovascular risk factors were found between the pSS group and controls. The pSS group had significantly lower GLS (P =.015) and GCS (P =.008) than the control group. Multiple linear regression analysis indicated that GCS was significantly associated with Raynaud's phenomenon (P =.015), focus score ≥2 (P =.032), and total ESSDAI score ≥8 (P =.029). Conclusion: FT‐CMR can reveal subclinical LV regional dysfunction in patients with pSS without cardiovascular disease. Furthermore, patients with pSS and Raynaud's phenomenon, a focus score ≥2, or an ESSDAI score ≥8 were considered to be at high risk for myocardial dysfunction. [ABSTRACT FROM AUTHOR]

Introduction: The low frequency of ectopic germinal center in labial salivary glands of patients with human T‐cell leukemia virus type 1 (HTLV‐1) antibody‐positive Sjögren's syndrome (SS) suggests that HTLV‐1 has some effects on follicular dendritic cells (FDCs). Methods: We used flow cytometry, immunofluorescence, and enzyme‐linked immunosorbent assays (ELISAs) to investigate the direct effect of HTLV‐1 on B‐cell activating factors produced by established FDC like cells obtained from excised human tonsils. We then measured the serum B‐cell activating factor (BAFF) and C‐X‐C motif ligand (CXCL) 13 concentrations of the HTLV‐1‐seropositive SS patients and the HTLV‐1‐seronegative SS patients by ELISA. Results: Among the 31 isolated specimens, 22 showed morphological characteristics of FDCs. Day 2‐cultured specimens showed expressions of CD14, CD23, CD40, intracellular adhesion molecule‐1 (ICAM‐1), and vascular cell adhesion molecule‐1. After 2 weeks, 12 of these specimens expressed ICAM‐1, FDC, and fibroblast cell marker. Intracellular BAFF and CXCL13 were constitutively expressed regardless of stimulation. After direct coculture with the HTLV‐1‐infected T‐cell line HCT‐5 or MT‐2, the BAFF and CXCL13 expressions on the FDC‐like cells were decreased in accord with the increased number of HCT‐5 and MT‐2 cells with styliform change and without HTLV‐1 Gag protein expression. Interferons upregulated the concentration of BAFF (but not CXCL13) in the culture supernatant, which showed a declining trend under the presence of HCT‐5 or MT‐2. The serum concentrations of BAFF and CXCL13 in the HTLV‐1‐seropositive SS patients were lower than those of the HTLV‐1 seronegative SS patients. Conclusions: HTLV‐1 partially inhibited the BAFF and CXCL13 expressions of established FDC‐like cells. [ABSTRACT FROM AUTHOR]

Objective: To clarify the significance of immunometabolism in systemic lupus erythematosus (SLE), and to determine the effect of calcium/calmodulin‐dependent protein kinase 4 (CaMK4) on T cell metabolism. Methods: Metabolomic profiling was performed using capillary electrophoresis mass spectrometry in naive T cells from MRL/lpr mice treated with anti‐CD3/CD28 antibodies in the absence or presence of a CaMK4 inhibitor (KN‐93). The expression of GLUT1 and CaMK4 in CD4+ T cells from healthy controls (n = 16), patients with inactive SLE (n = 13), and patients with active SLE (n = 14) was examined by flow cytometry and quantitative polymerase chain reaction. In vitro experiments were performed to determine the effect of KN‐93 on the expression of GLUT1 during Th17 cell differentiation in T cells from patients with SLE. Results: CaMK4 inhibition significantly decreased the levels of glycolytic intermediates such as glucose‐6‐phosphate, fructose‐6‐phosphate, fructose‐1,6‐diphosphate, pyruvate, and lactate (P < 0.05), whereas it did not affect the levels of the pentose phosphate pathway intermediates such as 6‐phospho‐d‐gluconate, ribulose‐5‐phosphate, ribose‐5‐phosphate, and phosphoribosyl pyrophosphate. The expression levels of GLUT1 and CaMK4 in effector memory CD4+ T cells were significantly higher in patients with active SLE compared to healthy controls (P < 0.01 and P < 0.05, respectively) and patients with inactive SLE (P < 0.05 and P < 0.01, respectively). A functional analysis revealed that CaMK4 inhibition decreased the expression of GLUT1 during Th17 cell differentiation (P < 0.01), followed by a reduction of interleukin‐17 (IL‐17) production (P < 0.05). Conclusion: The results of the study indicate that the activity of CaMK4 could be responsible for glycolysis, which contributes to the production of IL‐17, and CaMK4 may contribute to aberrant expression of GLUT1 in T cells from patients with active SLE. [ABSTRACT FROM AUTHOR]

Aim: We conducted this retrospective study to identify objective and comprehensive diagnostic criteria for early‐stage rheumatoid arthritis (RA) that are based on ultrasound (US) and serologic findings. Method: From August 2014 to May 2016, we recruited 216 consecutive patients at Hospital 1 and 223 consecutive patients at Hospital 2 who were suspected to have RA and underwent US of bilateral hands. In the US of bilateral hands from 22 sites, the findings obtained by grayscale and power Doppler (PD) assessments were each graded on a semi‐quantitative scale from 0 to 3. We also examined the assessment of the novel outcome measures in rheumatology (OMERACT)‐European League Against Rheumatism (EULAR) combined power Doppler ultrasound score (ie the cPD score) and the Global OMERACT‐EULAR Synovitis Score. We used the US findings and the combination of US and serologic findings to evaluate the diagnostic performance of these modalities. Results: Seventy patients (32.4%) at Hospital 1 and 59 patients (26.5%) at Hospital 2 were diagnosed as having RA. The best‐balanced diagnostic performance at each hospital was achieved using a combination, such as (1) the presence of PD grade ≥2 articular synovitis or (2) the presence of PD grade ≥1 articular synovitis and serologic positivity, as well as by using (1) the presence of cPD grade = 3 or (2) a cPD grade ≥2 and serologic positivity. Conclusion: The combination of a PD assessment or the cPD score with the measurement of autoantibodies of rheumatoid factor and/or anti‐cyclic citrullinated peptide antibodies can accurately identify patients with early‐stage RA. [ABSTRACT FROM AUTHOR]

Objectives: We investigated the impact of renal involvement at diagnosis on the prognosis of patients with antineutrophilic cytoplasmic antibody (ANCA)‐associated vasculitis (AAV). Methods: The relationship between renal involvement at diagnosis, clinical variables at diagnosis, and prognosis (including relapse episodes, initiation of dialysis, and death) was examined in 101 Japanese patients with AAV. Results: Sixty‐eight patients had renal involvement at diagnosis. The renal‐involvement patients had significantly higher ages at diagnosis, significantly lower hemoglobin levels, and significantly lower platelet levels. They had significantly lower C3 levels, but showed no significant difference in C4 levels. Overall survival rate was significantly worse in patients with than in patients without renal involvement (P = 0.003, log‐rank test). Multivariable analysis using a logistic regression model demonstrated that C3 contributed to dialysis initiation: odds ratio (per 10 mg/dL of C3): 0.68; range: 0.49‐0.90; P = 0.007. A Cox proportional hazard model revealed that the C3 level and age at diagnosis contributed significantly to overall survival: hazard ratio (per 10 mg/dL of C3) 0.81, range 0.69‐0.95, P = 0.011; 1.08, 1.02‐1.15, P = 0.013, respectively. Renal involvement did not contribute significantly to overall survival. Patients with C3 levels ≥100 mg/dL had a better survival rate than patients with C3 levels <100 mg/dL. Conclusions: Although patients with renal involvement had higher ages, lower C3 levels at diagnosis, and poorer prognoses, multivariable analysis demonstrated that the C3 level and age at diagnosis, but not renal involvement, contributed significantly to overall survival. Our results demonstrate the relationship between C3 hidden behind renal involvement and AAV prognosis. [ABSTRACT FROM AUTHOR]

Objective: To explore the variables associated with initial favorable power Doppler (PD) ultrasound (US) response induced by biologic and targeted synthetic disease-modifying antirheumatic drugs (b/tsDMARDs) in patients with rheumatoid arthritis (RA).Methods: We have been prospectively investigating the course of active RA patients using US after the introduction of b/tsDMARDs in the Kyushu region of Japan since June 2013. A total of 150 patients have completed the first 6 months of observation at present and have been evaluated. US was assessed in 22 joints of bilateral hands using gray-scale and PD images on a scale from 0-3. The sum of these scores was used as the indicator of US disease activity. We defined PD remission as attaining a total PD score of 0 at 6 months and investigated the associated variables by multivariate logistic regression analysis.Results: The total PD and gray-scale scores and the clinical composite measures significantly improved at 6 months, whereas these reductions were less in bDMARD switchers as compared with bDMARD-naive patients. Multivariate logistic regression analysis showed that short disease duration, the absence of any previous use of bDMARDs, and low total PD scores at baseline were independent predictors of PD remission at 6 months.Conclusion: This present prospective US cohort has for the first time shown the variables that are associated with initial PD response to b/tsDMARDs. [ABSTRACT FROM AUTHOR]

Abstract: Objective: To determine the effect of leuprolide acetate, a synthetic gonadotropin‐releasing hormone analog (GnRH‐a) on ovarian function preservation in systemic lupus erythematosus (SLE) patients treated with cyclophosphamide (CYC) in clinical practice. Methods: We enrolled 30 premenopausal female SLE patients who fulfilled the 1997 American College of Rheumatology revised criteria and were treated with intravenous CYC (IVCY) in 2008–2017. We used Kaplan–Meier survival estimates to compare the GnRH‐a‐treated patients and those not treated with GnRH‐a as controls. We performed Cox regression analyses to identify factors associated with premature ovarian failure (POF), incidences of cardiovascular events, strokes and osteoporosis after IVCY therapy. Results: After a mean follow‐up of 41 months, POF developed in one of the 16 GnRH‐a‐treated patients (6%) versus seven of the 14 controls (50%). Significantly improved cumulative ovarian protection over time was observed in the GnRH‐a‐treated group (P = 0.030). The hazard model analysis showed that treatment with GnRH‐a during IVCY therapy is an independent factor associated with POF after IVCY therapy (adjusted hazards ratio = 0.12, 95% CI 0.01–0.67, P = 0.013) but not incidences of cardiovascular events, strokes or osteoporosis. Conclusion: The combined use of GnRH‐a with IVCY therapy was associated with a significant reduction of POF among premenopausal women with SLE, suggesting that the addition of GnRH‐a can be a strategy to prevent POF among premenopausal women with SLE after IVCY therapy. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 46-year-old woman, who had a history of general edema and dyspnea. Topics discussed include the soft-tissue edema revealed in a computed tomography (CT) scan, the diagnosis of protein-losing entropathy (PLE), and the patient's resistance to corticosteroid therapy. Also mentioned is the use of immunosuppressant therapy to treat the patient.

Objectives The risk factors of adverse events during a number of currently used treatments for rheumatoid arthritis ( RA) in elderly patients were examined. Methods A retrospective observational study was conducted for 300 elderly RA patients registered in December 2009 at Sasebo Chuo Hospital, Japan, and the adverse events during the treatments for RA were assessed. Results The average age of the patients was 74.3 ± 5.8 years. The Steinbrocker stage was IV in almost one-half of the patients. Methotrexate ( MTX) was used in 54.0% of patients and biologics in 23.0% of patients. Adverse events occurred in 103 patients (34.3%). The most common adverse events were infections (46.6%), including pneumonia (21.4%). Multiple logistic analyses revealed that the factors significantly related to infection were advanced Steinbrocker stage and the existence of respiratory diseases, and that of pneumonia was the existence of diabetes mellitus ( DM). Conclusions The elderly RA patients with advanced stage, respiratory diseases or DM should be monitored for infections, including pneumonia, carefully during treatment. [ABSTRACT FROM AUTHOR]

Objective Kidney podocytes and their slit diaphragms prevent urinary protein loss. T cells from patients with systemic lupus erythematosus display increased expression of calcium/calmodulin-dependent protein kinase IV (CaMKIV). The present study was undertaken to investigate the role of CaMKIV in podocyte function in lupus nephritis (LN). Methods We treated kidney podocytes with IgG derived from healthy individuals or patients with LN and then analyzed gene expression using a DNA microarray. The localization of IgG in podocytes was analyzed by immunofluorescence staining, with or without silencing of neonatal Fc receptor (FcRn). In addition, we silenced CAMK4 in podocytes and analyzed the expression of selected genes. We also examined the expression of CD86 in kidney podocytes from MRL/ lpr, MRL/ lpr.camkiv-/ -, and MRL/ MPJ mice by in situ hybridization. Results We found that exposure of podocytes to IgG resulted in entry of IgG into the cytoplasm. IgG entered podocytes via the FcRn because less IgG was found in the cytoplasm of podocytes treated with FcRn small interfering RNA. DNA microarray studies of podocytes exposed to LN-derived IgG revealed up-regulation of genes related to the activation of immune cells or podocyte damage. Interestingly, CD86 expression decreased after silencing CAMK4 in podocytes. Also, in situ hybridization experiments showed that the expression of CD86 was reduced in podocytes from MRL/ lpr.camkiv-/ - mice. Conclusion LN-derived IgG enters podocytes and up-regulates CAMK4, which is followed by increased expression of genes known to be linked to podocyte damage and T cell activation. Targeted inhibition of CAMK4 in podocytes may prove to be clinically useful in patients with LN. [ABSTRACT FROM AUTHOR]

Objective To investigate the relationship between the intercellular transmission efficiency of human T-lymphotropic virus type I ( HTLV-I) and the signaling involved in actin polymerization during cytoskeletal reorganization in a comparative study of HTLV-I-infected T-cell lines derived from an HTLV-I-associated myelopathy/tropical spastic paraparesis ( HAM/ TSP) patient or an HTLV-I carrier. Methods HCT-5 and TL-Su cells derived from an HAM/TSP patient and an HTLV-I carrier, respectively, were used as HTLV-I-infected T-cell lines. After co-cultivation of each HTLV-I-infected T-cell line with H9/K30 luc reporter cells, the relative luc activities were calculated to analyze the efficiency of intercellular transmission of HTLV-I. The intracellular levels of cyclic adenosine monophosphate (c AMP) or cyclic guanosine monophosphate (c GMP) were measured in enzyme-linked immunoassays. The expression of phosphorylated vasodilator-stimulated phosphoprotein (p-VASP) was analyzed by western blotting. Results Treatment of HCT-5 cells with latrunculin B, an inhibitor of actin polymerization, significantly suppressed the relative luc activity. Western blotting analysis of HCT-5 cells treated with the adenylyl cyclase activator forskolin showed upregulation of p-VASP, with a concomitant and significant increase in the intracellular c AMP concentration. Furthermore, the relative luc activity was significantly decreased. The intracellular c AMP, but not c GMP levels, were significantly lower in HCT-5 than in TL-Su. Vasodilator-stimulated phosphoprotein appeared less phosphorylated in HCT-5 than in TL-Su. The relative luc activity was significantly higher in HCT-5 than in TL-Su. Conclusions The intracellular c AMP concentration regulates the efficiency of intercellular HTLV-I transmission under the control of p-VASP expression, suggesting the intercellular transmission potential of HTLV-I-infected T cells of HAM/TSP patients is enhanced by downregulated intracellular c AMP levels. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented regarding a case of a 53-year old man with xerophthalmia and xerostamia, and adult T-cell leukemia (ATL) mimicking sicca symptoms (SS).

We describe a case of early repolarization syndrome in which augmented J waves were documented during an electrical storm associated with hypokalemia. The patient was referred to our hospital for therapy to treat recurrent ventricular fibrillation (VF). The 12-lead electrocardiogram showed giant J waves associated with hypokalemia during multiple episodes of VF. Although antiarrhythmic agents or deep sedation were not effective for the VF, an intravenous supplementation of potassium completely suppressed the VF with a reduction in the J-wave amplitude. Our report discusses the possible relationship between hypokalemia and VF in early repolarization syndrome. (PACE 2012; 35:e234-e238) [ABSTRACT FROM AUTHOR]

We describe a case of a myocardial infarction, in which prominent ischemic J waves were documented during recurrent ventricular fibrillation attacks. The patient was referred to our hospital to treat an out-of hospital cardiac arrest. Although the 12-lead electrocardiogram obtained just after the first cardioversion did not show any apparent J waves, a J wave-like steep downsloping type ST-segment elevation associated with q waves in the inferior leads was documented during multiple episodes of ventricular fibrillation. Our report revealed the appearance of J waves as an important marker for lethal arrhythmias in acute ischemia. (PACE 2012; 35:e27-e30) [ABSTRACT FROM AUTHOR]

This article describes the development and implementation of adaptive transit signal priority (TSP) on an actuated dual-ring traffic signal control system. After providing an overview of architecture design of the adaptive TSP system, the article presents an adaptive TSP optimization model that optimizes green splits for three consecutive cycles to minimize the weighted sum of transit vehicle delay and other traffic delay, considering the safety and other operational constraints under the dual-ring structure of signal control. The model is illustrated using a numerical example under medium and heavily congested situations. The findings from a field operational test are also reported to validate and demonstrate the developed TSP system. At a congested intersection, it is found that the average bus delay and average traffic delay along the bus movement direction were reduced by approximately 43% and 16%, respectively. Moreover, the average delay of cross-street traffic was increased by about 12%. [ABSTRACT FROM AUTHOR]

Mutations in the plectin gene ( PLEC) generally lead to epidermolysis bullosa simplex (EBS) associated with muscular dystrophy. It has been recently demonstrated that PLEC mutations can also cause a different clinical subtype, EBS associated with pyloric atresia (EBS-PA), which shows early lethality. Prenatal diagnosis (PND) of EBS-PA using mutation screening of PLEC has not been described. This study aimed to perform DNA-based PND for an EBS-PA family. The EBS-PA proband was compound-heterozygous for a paternal c.1350G>A splice-site mutation and a maternal p.Q305X nonsense mutation. Genomic DNA was obtained from amniocytes taken from an at-risk fetus of the proband's family. Direct sequencing and restriction enzyme digestion of polymerase chain reaction products from the genomic DNA were performed. Mutational analysis showed that the fetus harbored both pathogenic mutations, suggesting that the fetus was a compound-heterozygote and therefore affected with EBS-PA. The skin sample obtained by autopsy from the abortus confirmed the absence of plectin expression at the dermal-epidermal junction. This is the first successful DNA-based PND for an EBA-PA family. [ABSTRACT FROM AUTHOR]

Objective To verify whether magnetic resonance imaging (MRI)-proven joint injury is sensitive as compared with joint injury determined by physical examination. Methods MRI of the wrist and finger joints of both hands was examined in 51 early-stage rheumatoid arthritis (RA) patients by both plain and gadolinium diethylenetriaminepentaacetic acid-enhanced MRI. Synovitis, bone edema, and bone erosion (the latter two included as bone lesions at the wrist joints); metacarpophalangeal joints; and proximal interphalangeal joints were considered as MRI-proven joint injury. Japan College of Rheumatology-certified rheumatologists had given a physical examination just before the MRI study. The presence of tender and/or swollen joints in the same fields as MRI was considered as joint injury on physical examination. The association of MRI-proven joint injury with physical examination-proven joint injury was examined. Results A total of 1,110 sites were available to be examined. MRI-proven joint injury was found in 521 sites, whereas the other 589 sites were normal. Physical examination-proven joint injury was found in 305 sites, which was significantly low as compared with MRI-proven joint injury ( P = 1.1 × 10−12 versus MRI). Joint injury on physical examination was not found in 81.5% of the sites where MRI findings were normal. Furthermore, an association of the severity of MRI-proven joint injury with that of joint injury on physical examination was clearly demonstrated ( P = 1.6 × 10−15, rs = 0.469). Conclusion Our present data suggest that MRI is not only sensitive but accurately reflects the joint injury in patients with early-stage RA. [ABSTRACT FROM AUTHOR]

Abstract:We treated 12 patients with rheumatoid arthritis by filtration leukocytapheresis (FLCP) and evaluated its effect on leukocyte enzyme activities. We calculated the number of leukocytes removed and assessed the clinical response. We also evaluated the cellular enzyme activities of elastase and dipeptidylpeptidase IV (DPP IV). Out of 12 patients, 7 patients achieved 20% improvement for 4 weeks following FLCP. The FLCP treatment resulted in removal of 96% of granulocytes, 98% of monocytes, and 61% of lymphocytes. Granulocytes and monocytes with high elastase activity were effectively removed by FLCP. The elastase activity of granulocytes was increased 4 weeks after the last FLCP only in responders. On the other hand, the DPP IV activity of lymphocytes was low at 4 weeks after the last FLCP in responders. Modulation of leukocyte enzyme activities is one of the main effects of FLCP therapy and alteration of granulocytes, monocytes, and lymphocytes. [ABSTRACT FROM AUTHOR]

Background: Angiosarcomas arise in the scalp and face in the elderly, in association with chronic lymphedema (Stewart–Treves syndrome), and in irradiated areas. Rarely in these settings, angiosarcomas exhibit pure spindle cell phenotype. Methods: Herein, the clinicopathologic features of a 72-year-old-woman with spindle cell angiosarcoma are described. Results: A 72-year-old woman presented with numerous nodules and diffuse induration from the lower abdomen to the right buttock, corresponding to the area exposed to 60 Co-irradiation during treatment for cervical carcinoma 10 years earlier. Histopathological examination revealed inflitrative, atypical, spindle cells that labeled with antibodies to CD31, CD34, and factor VIII-related antigen. Ultrastructurally, these malignant spindle cells contained Weibel–Palade bodies. No features suggesting radiation dermatitis (sclerosis or bizarre, large fibroblasts) were identified, but lymphangiectases and widely spaced collagen bundles(lymphedema) were prominent in the skin surrounding the angiosarcoma. Computed tomographic scan of the abdomen highlighted this histologic finding by demonstrating tumor masses limited to areas of lymphedema. Treatment with intravenous and local injections of recombinant interleukin 2 (rIL 2) followed by electron beam irradiation were initially effective with tumor remission for 2 months. However, the recurrent tumor did not respond to a second course of one-shot injection of rIL 2 through the abdominal aorta and the patient succumbed to her angiosarcoma 19 months after diagnosis. Conclusions: Radiation-induced lymphedema may be a factor in angiosarcoma associated with radiotherapy. [ABSTRACT FROM AUTHOR]

Abstract:We evaluated the relationship between the clinical benefit of filtration leukocytapheresis (LCP) and the number of removed leukocytes in patients with rheumatoid arthritis (RA). LCP was performed in 31 drug-resistant RA patients. LCP was carried out 3 times with 1 week separating each session. Assessment of RA before and after LCP showed a substantial and rapid improvement in tender joint counts, swollen joint counts, and patients' and physicians' assessments. Careful analysis indicated that 19 of 31 patients with RA showed ≥20% improvement following LCP therapy. The number of leukocytes in the peripheral blood significantly decreased during each session of LCP. However, there was no significant decrease in the number of circulating blood cells during the study period. No adverse reactions or complications were noted. There was no significant difference in any indices of clinical activity and the removal rates of leukocytes between responders and nonresponders. The total numbers of removed lymphocytes in responders were significantly higher than those in nonresponders (responders 64.1 × 108 versus nonresponders 50.7 ×108 , p < 0.05). The relationship between clinical effectiveness and the number of removed granulocytes and monocytes was not statistically significant. Our results suggest that filtration LCP to remove leukocytes from the peripheral blood, especially lymphocytes, exerts an immunomodulatory effect in patients with RA. [ABSTRACT FROM AUTHOR]

Aims/Background: This study was undertaken in order to characterize the liver injury induced by transcatheter arterial chemoembolization therapy (TACE) for hepatocellular carcinoma (HCC) and to elucidate mechanisms involved in the growth of mononuclear phagocytes in injured human liver in vivo. Patients and Methods: The serum levels of macrophage-colony stimulating factor (M-CSF) along with clinical parameters were examined in 43 patients with HCC who underwent TACE. Ten patients who underwent angiography alone served as controls. Results: Serum M-CSF increased and peaked on the third day after TACE showing significant correlations ( p<0.001, respectively) with the increases in serum alanine aminotransferase (ALT) and type IV collagen-7S (IVcol-7S). The lipopolysaccharide-stimulated production of interleukin (IL)-1β, IL-6, and tumor necrosis factor (TNF)-α in peripheral whole blood increased and peaked on the first or on the third day after TACE. In effective cases of TACE, significantly ( p<0.05) greater increases in serum M-CSF were noted as compared with those in ineffective cases. Discussion: The serum levels of M-CSF increased after TACE in correlation with hepatic inflammation and necrosis and increased production of IL-1β, TNF-α, and IL-6 in peripheral whole blood. These results suggest a mechanism by which hepatic injury enhances the production of M-CSF via a cytokine cascade, which results in the proliferation of liver macrophages in vivo. [ABSTRACT FROM AUTHOR]

Junctional epidermolysis bullosa associated with pyloric atresia (JEB-PA) is one of the most severe inherited skin diseases, characterized by generalized blister formation and occlusion of the pylorus at birth. Most JEB-PA patients have mutations in the gene encoding β4 integrin ( ITGB4). No recurrent mutations in ITGB4 have been described as having founder effects. We collected three JEB-PA families with c.1938delC in ITGB4. Haplotype analysis using single nucleotide polymorphism markers throughout ITGB4 suggested one rare haplotype (2.8% of the Han Chinese and ethnic Japanese populations) in all alleles with c.1938delC. The parents of one of the three families sought prenatal diagnosis for a subsequent pregnancy. We succeeded in performing prenatal exclusion of JEB-PA using the foetal genomic DNA. Our study clearly demonstrated that recurrent c.1938delC in ITGB4 is a founder mutation in JEB-PA patients, and that genotyping of the mutation can be utilized for prenatal diagnosis of JEB-PA. [ABSTRACT FROM AUTHOR]