A patient with hypereosinophilic syndrome that manifested with acquired hemophilia and elevated IgG4: a case report

<p>Abstract</p> <p>Introduction</p> <p>Hypereosinophilic syndrome is defined as a prolonged state (more than six months) of eosinophilia (greater than 1500 cells/μL), without an apparent etiology and with end-organ damage. Hypereosinophilic syndrome can cause coagulation abnormalities. Among hypereosinophilic syndrome types, the lymphocytic variant (lymphocytic hypereosinophilic syndrome) is derived from a monoclonal proliferation of T lymphocytes. Here, we describe the case of a patient with lymphocytic hypereosinophilic syndrome who presented with a coagulation abnormality. To the best of our knowledge, this is the first such report including a detailed clinical picture and temporal cytokine profile.</p> <p>Case presentation</p> <p>A 77-year-old Japanese man presented to our facility with massive hematuria and hypereosinophilia (greater than 2600 cells/μl). His eosinophilia first appeared five years earlier when he developed femoral artery occlusion. He manifested with multiple hematomas and prolonged activated partial thromboplastin time. His IgG4 level was remarkably elevated (greater than 2000 mg/dL). Polymerase chain reaction tests of peripheral blood and bone marrow identified lymphocytic hypereosinophilic syndrome. His prolonged activated partial thromboplastin time was found to be due to acquired hemophilia. Glucocorticoids suppressed both the hypereosinophilia and coagulation abnormality. However, tapering of glucocorticoids led to a relapse of the coagulation abnormality alone, without eosinophilia. Tumor necrosis factor α, interleukin-5, and/or eotaxin-3 may have caused the hypereosinophilia, and interleukin-10 was correlated with the coagulation abnormality.</p> <p>Conclusions</p> <p>To the best of our knowledge, this is the first case in which lymphocytic hypereosinophilic syndrome and IgG4-related disease have overlapped. In addition, our patient is only the second case of hypereosinophilic disease that manifested with acquired hemophilia. Our patient relapsed with the coagulation abnormality alone, without eosinophilia. This report shows that the link between eosinophilia, IgG4, and clinical manifestations is not simple and provides useful insight into the immunopathology of hypereosinophilic syndrome and IgG4-related disease.</p

A NORTHERN SKY SURVEY FOR POINT-LIKE SOURCES OF EeV NEUTRAL PARTICLES WITH THE TELESCOPE ARRAY EXPERIMENT

We report on the search for steady point-like sources of neutral particles around 10(18) eV between 2008 and 2013 May with the scintillator SD of the Telescope Array experiment. We found overall no significant point-like excess above 0.5 EeV in the northern sky. Subsequently, we also searched for coincidence with the Fermi bright Galactic sources. No significant coincidence was found within the statistical uncertainty. Hence, we set an upper limit on the neutron flux that corresponds to an averaged flux of 0.07 km(-2) yr(-1) for E &gt; 1 EeV in the northern sky at the 95% confidence level. This is the most stringent flux upper limit in a northern sky survey assuming point-like sources. The upper limit at the 95% confidence level on the neutron flux from Cygnus X-3 is also set to 0.2 km(-2) yr(-1) for E &gt; 0.5 EeV. This is an order of magnitude lower than previous flux measurementsopen0

Measurement of the proton-air cross section with Telescope Array&apos;s Middle Drum detector and surface array in hybrid mode

In this work we are reporting on the measurement of the proton-air inelastic cross section sigma(inel)(p-air) using the Telescope Array detector. Based on the measurement of the sigma(inel)(p-air), the proton-proton cross section sigma(p-p) value is also determined at root s = 95(-8)(+5) TeV. Detecting cosmic ray events at ultrahigh energies with the Telescope Array enables us to study this fundamental parameter that we are otherwise unable to access with particle accelerators. The data used in this report are the hybrid events observed by the Middle Drum fluorescence detector together with the surface array detector collected over five years. The value of the sigma(inel)(p-air) is found to be equal to 567.0 +/- 70.5[Stat](-25)(+29)[Sys] mb. The total proton-proton cross section is subsequently inferred from Glauber formalism and the Block, Halzen and Stanev QCD inspired fit and is found to be equal to 170(-44)(+48)[Stat](-17)(+19)[Sys] mb.open20

Indications of intermediate-scale anisotropy of cosmic rays with energy greater than 57 EeV in the northern sky measured with the surface detector of the Telescope Array experiment

We have searched for intermediate-scale anisotropy in the arrival directions of ultrahigh-energy cosmic rays with energies above 57 EeV in the northern sky using data collected over a 5 yr period by the surface detector of the Telescope Array experiment. We report on a cluster of events that we call the hotspot, found by oversampling using 20?? radius circles. The hotspot has a Li-Ma statistical significance of 5.1??, and is centered at R.A. = 146.??7, decl. = 43.??2. The position of the hotspot is about 19?? off of the supergalactic plane. The probability of a cluster of events of 5.1?? significance, appearing by chance in an isotropic cosmic-ray sky, is estimated to be 3.7 ?? 10-4 (3.4??).open2

CORRELATIONS OF THE ARRIVAL DIRECTIONS OF ULTRA-HIGH ENERGY COSMIC RAYS WITH EXTRAGALACTIC OBJECTS AS OBSERVED BY THE TELESCOPE ARRAY EXPERIMENT

We search for correlations between the positions of extragalactic objects and the arrival directions of ultra-high energy cosmic rays (UHECRs) with primary energy E ??? 40 EeV as observed by the surface detector array of the Telescope Array (TA) experiment during the first 40 months of operation. We examine several public astronomical object catalogs, including the Veron-Cetty and Veron catalog of active galactic nuclei. We count the number of TA events correlated with objects in each catalog as a function of three parameters: the maximum angular separation between a TA event and an object, the minimum energy of the events, and the maximum redshift of the objects. We determine the combination of these parameters that maximizes the correlations, and we calculate the probability of having the same levels of correlations from an isotropic distribution of UHECR arrival directions. No statistically significant correlations are found when penalties for scanning over the above parameters and for searching in several catalogs are taken into account.open4

Update on the Combined Analysis of Muon Measurements from Nine Air Shower Experiments

Over the last two decades, various experiments have measured muon densities in extensive air showers over several orders of magnitude in primary energy. While some experiments observed differences in the muon densities between simulated and experimentally measured air showers, others reported no discrepancies. We will present an update of the meta-analysis of muon measurements from nine air shower experiments, covering shower energies between a few PeV and tens of EeV and muon threshold energies from a few 100 MeV to about 10GeV. In order to compare measurements from different experiments, their energy scale was cross-calibrated and the experimental data has been compared using a universal reference scale based on air shower simulations. Above 10 PeV, we find a muon excess with respect to simulations for all hadronic interaction models, which is increasing with shower energy. For EPOS-LHC and QGSJet-II.04 the significance of the slope of the increase is analyzed in detail under different assumptions of the individual experimental uncertainties