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9. Determinants of lenalidomide response with or without erythropoiesis-stimulating agents in myelodysplastic syndromes:the HOVON89 trial

Author

van de Loosdrecht, A. A., Cremers, E. M.P., Alhan, C., Duetz, C., in ’t Hout, F. E.M., Visser-Wisselaar, H. A., Chitu, D. A., Verbrugge, A., Cunha, S. M., Ossenkoppele, G. J., Janssen, J. J.W.M., Klein, S. K., Vellenga, E., Huls, G. A., Muus, P., Langemeijer, S. M.C., de Greef, G. E., te Boekhorst, P. A.W., Raaijmakers, M. H.G., van Marwijk Kooy, M., Legdeur, M. C., Wegman, J. J., Deenik, W., de Weerdt, O., van Maanen-Lamme, T. M., Jobse, P., van Kampen, R. J.W., Beeker, A., Wijermans, P. W., Biemond, B. J., Tanis, B. C., van Esser, J. W.J., Schaar, C. G., Noordzij-Nooteboom, H. S., Jacobs, E. M.G., de Graaf, A. O., Jongen-Lavrencic, M., Stevens-Kroef, M. J.P.L., Westers, T. M., Jansen, J. H., van de Loosdrecht, A. A., Cremers, E. M.P., Alhan, C., Duetz, C., in ’t Hout, F. E.M., Visser-Wisselaar, H. A., Chitu, D. A., Verbrugge, A., Cunha, S. M., Ossenkoppele, G. J., Janssen, J. J.W.M., Klein, S. K., Vellenga, E., Huls, G. A., Muus, P., Langemeijer, S. M.C., de Greef, G. E., te Boekhorst, P. A.W., Raaijmakers, M. H.G., van Marwijk Kooy, M., Legdeur, M. C., Wegman, J. J., Deenik, W., de Weerdt, O., van Maanen-Lamme, T. M., Jobse, P., van Kampen, R. J.W., Beeker, A., Wijermans, P. W., Biemond, B. J., Tanis, B. C., van Esser, J. W.J., Schaar, C. G., Noordzij-Nooteboom, H. S., Jacobs, E. M.G., de Graaf, A. O., Jongen-Lavrencic, M., Stevens-Kroef, M. J.P.L., Westers, T. M., and Jansen, J. H.

Abstract

A randomized phase-II study was performed in low/int-1 risk MDS (IPSS) to study efficacy and safety of lenalidomide without (arm A) or with (arm B) ESA/G-CSF. In arm B, patients without erythroid response (HI-E) after 4 cycles received ESA; G-CSF was added if no HI-E was obtained by cycle 9. HI-E served as primary endpoint. Flow cytometry and next-generation sequencing were performed to identify predictors of response. The final evaluation comprised 184 patients; 84% non-del(5q), 16% isolated del(5q); median follow-up: 70.7 months. In arm A and B, 39 and 41% of patients achieved HI-E; median time-to-HI-E: 3.2 months for both arms, median duration of-HI-E: 9.8 months. HI-E was significantly lower in non-del(5q) vs. del(5q): 32% vs. 80%. The same accounted for transfusion independency-at-week 24 (16% vs. 67%), but similar in both arms. Apart from presence of del(5q), high percentages of bone marrow lymphocytes and progenitor B-cells, a low number of mutations, absence of ring sideroblasts, and SF3B1 mutations predicted HI-E. In conclusion, lenalidomide induced HI-E in patients with non-del(5q) and del(5q) MDS without additional effect of ESA/G-CSF. The identified predictors of response may guide application of lenalidomide in lower-risk MDS in the era of precision medicine. (EudraCT 2008-002195-10).

Published
2024

10. The first spectroscopic IR reverberation programme on Mrk 509

Author

Mitchell, J. A. J., Ward, M. J., Kynoch, D., Santisteban, J. V. Hernández, Horne, K., Pott, J. -U., Esser, J., Mercatoris, P., Packham, C., Ferland, G. J., Lawrence, A., Fischer, T., Barth, A. J., Villforth, C., Winkler, H., Mitchell, J. A. J., Ward, M. J., Kynoch, D., Santisteban, J. V. Hernández, Horne, K., Pott, J. -U., Esser, J., Mercatoris, P., Packham, C., Ferland, G. J., Lawrence, A., Fischer, T., Barth, A. J., Villforth, C., and Winkler, H.

Abstract

Near IR spectroscopic reverberation of Active Galactic Nuclei (AGN) potentially allows the infrared (IR) broad line region (BLR) to be reverberated alongside the disc and dust continua, while the spectra can also reveal details of dust astro-chemistry. Here, we describe results of a short pilot study (17 near-IR spectra over a 183 d period) for Mrk 509. The spectra give a luminosity-weighted dust radius of $\langle R_{\mathrm{d,lum}} \rangle = 186 \pm 4$ light-days for blackbody (large grain dust), consistent with previous (photometric) reverberation campaigns, whereas carbon and silicate dust give much larger radii. We develop a method of calibrating spectral data in objects where the narrow lines are extended beyond the slit width. We demonstrate this by showing our resultant photometric band light curves are consistent with previous results, with a hot dust lag at >40 d in the K band, clearly different from the accretion disc response at <20 d in the z band. We place this limit of 40 d by demonstrating clearly that the modest variability that we do detect in the H and K band does not reverberate on time-scales of less than 40 d. We also extract the Pa$\beta$ line light curve, and find a lag which is consistent with the optical BLR H$\beta$ line of $\sim$70-90 d. This is important as direct imaging of the near-IR BLR is now possible in a few objects, so we need to understand its relation to the better studied optical BLR., Comment: 16 pages, 12 figures

Published
2024
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11. Expected Performance of the EUSO-SPB2 Fluorescence Telescope

Author

Filippatos, G., Battisti, M., Bertaina, M., Bisconti, F., Esser, J., Heaton, C., Osteria, G., Sarazin, F., Wiencke, L., Filippatos, G., Battisti, M., Bertaina, M., Bisconti, F., Esser, J., Heaton, C., Osteria, G., Sarazin, F., and Wiencke, L.

Abstract

The Extreme Universe Space Observatory Supper Pressure Balloon 2 (EUSO-SPB2) is under development, and will prototype instrumentation for future satellite-based missions, including the Probe of Extreme Multi-Messenger Astrophysics (POEMMA). EUSO-SPB2 will consist of two telescopes. The first is a Cherenkov telescope (CT) being developed to identify and estimate the background sources for future below-the-limb very high energy (E>10 PeV) astrophysical neutrino observations, as well as above-the-limb cosmic ray induced signals (E>1 PeV). The second is a fluorescence telescope (FT) being developed for detection of Ultra High Energy Cosmic Rays (UHECRs). In preparation for the expected launch in 2023, extensive simulations tuned by preliminary laboratory measurements have been preformed to understand the FT capabilities. The energy threshold has been estimated at $10^{18.2}$ eV, and results in a maximum detection rate at $10^{18.6}$ eV when taking into account the shape of the UHECR spectrum. In addition, onboard software has been developed based on the simulations as well as experience with previous EUSO missions. This includes a level 1 trigger to be run on the computationally limited flight hardware, as well as a deep learning based prioritization algorithm in order to accommodate the balloon's telemetry budget. These techniques could also be used later for future, space-based missions., Comment: ICRC 2021

Published
2021
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14. Vernetzung und Kooperation ehrenamtlicher und akademischer Forschung im Rahmen des nationalen Biodiversitätsmonitorings: Herausforderungen und Lösungsstrategien. Networking and cooperation of voluntary and academic research within the framework of national biodiversity monitoring – challenges and solution strategies

Author

Sommerwerk, N., Geschke, J., Schliep, R., Esser, J., Glöckler, F., Grossart, H.-P., Hand, R., Kiefer, S., Kimmig, S., Koch, A., Kühn, Elisabeth, Larondelle, N., Lehmann, G., Munzinger, S., Rödl, T., Werner, D., Wessel, M., Vohland, K., Sommerwerk, N., Geschke, J., Schliep, R., Esser, J., Glöckler, F., Grossart, H.-P., Hand, R., Kiefer, S., Kimmig, S., Koch, A., Kühn, Elisabeth, Larondelle, N., Lehmann, G., Munzinger, S., Rödl, T., Werner, D., Wessel, M., and Vohland, K.

Abstract

Die systematische Erfassung und Analyse von Biodiversitätsdaten und Ökosystemleistungen ist unerlässlich. Sie trägt zum Verständnis von Ursachen des Biodiversitätsverlusts bei und wird zur Analyse von Trends sowie zur Evaluierung von Interventionsmaßnahmen und politischen Strategien benötigt. Ehrenamtliche Datenerhebungen und wissenschaftliche Analysen spielen bereits seit Jahrzehnten eine tragende Rolle bei der behördlichen Berichterstattung. In diesem Artikel diskutieren wir, welche Schwachstellen und Optimierungsmöglichkeiten dabei in der Zusammenarbeit zwischen ehrenamtlicher und akademischer Forschung existieren. Eine effiziente und vertrauensvolle Zusammenarbeit an dieser Schnittstelle ist von grundlegender Bedeutung, insbesondere, wenn die auf freiwilliger Basis erhobenen Daten in der akademischen Forschung zu Biodiversität und Naturschutz genutzt werden sollen. Wir stellen zentrale Handlungsfelder und konkrete, praxisrelevante Maßnahmen vor, die wesentlich zur Verbesserung dieser Zusammenarbeit und dem Gelingen des nationalen Biodiversitätsmonitorings beitragen können. Dazu gehören ein gemeinsames Verständnis und Koordination des nationalen Biodiversitätsmonitorings, die Bereitstellung von Ressourcen zur Unterstützung nicht akademischer Forschung, Dateninfrastrukturen und Visualisierungstools, aber auch Bildungsanstrengungen zum Beispiel in Vereinen und Schulen. Networking and cooperation of voluntary and academic research within the framework of national biodiversity monitoring – challenges and solution strategies The systematic collection and analysis of biodiversity data and ecosystem services is essential. It contributes to the understanding of the causes of biodiversity loss and is needed to analyse trends and evaluate intervention measures and policies. Data collection by volunteers and scientific analyses have played a key role in government reporting for decades. In this article, we discuss weaknesses in the cooperation between volunteers and formal

Published
2021

15. Expected Performance of the EUSO-SPB2 Fluorescence Telescope

Author

Filippatos, G., Battisti, M., Bertaina, M., Bisconti, F., Esser, J., Heaton, C., Osteria, G., Sarazin, F., Wiencke, L., Filippatos, G., Battisti, M., Bertaina, M., Bisconti, F., Esser, J., Heaton, C., Osteria, G., Sarazin, F., and Wiencke, L.

Abstract

The Extreme Universe Space Observatory Supper Pressure Balloon 2 (EUSO-SPB2) is under development, and will prototype instrumentation for future satellite-based missions, including the Probe of Extreme Multi-Messenger Astrophysics (POEMMA). EUSO-SPB2 will consist of two telescopes. The first is a Cherenkov telescope (CT) being developed to identify and estimate the background sources for future below-the-limb very high energy (E>10 PeV) astrophysical neutrino observations, as well as above-the-limb cosmic ray induced signals (E>1 PeV). The second is a fluorescence telescope (FT) being developed for detection of Ultra High Energy Cosmic Rays (UHECRs). In preparation for the expected launch in 2023, extensive simulations tuned by preliminary laboratory measurements have been preformed to understand the FT capabilities. The energy threshold has been estimated at $10^{18.2}$ eV, and results in a maximum detection rate at $10^{18.6}$ eV when taking into account the shape of the UHECR spectrum. In addition, onboard software has been developed based on the simulations as well as experience with previous EUSO missions. This includes a level 1 trigger to be run on the computationally limited flight hardware, as well as a deep learning based prioritization algorithm in order to accommodate the balloon's telemetry budget. These techniques could also be used later for future, space-based missions., Comment: ICRC 2021

Published
2021
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18. Suppression of myocardial glucose metabolism in FDG PET/CT: impact of dose variation in heparin bolus pre-administration

Author

MS Radiologie, Researchgr. Nucleaire Geneeskunde, Cancer, Scholtens, A M, van den Berk, A M, van der Sluis, N L, Esser, J P, Lammers, G K, de Klerk, J M H, Lam, M G E H, Verberne, H J, MS Radiologie, Researchgr. Nucleaire Geneeskunde, Cancer, Scholtens, A M, van den Berk, A M, van der Sluis, N L, Esser, J P, Lammers, G K, de Klerk, J M H, Lam, M G E H, and Verberne, H J

Published
2020

19. Optical continuum photometric reverberation mapping of the Seyfert-1 galaxy Mrk509

Author

Nuñez, F. Pozo, Gianniotis, N., Blex, J., Lisow, T., Chini, R., Polsterer, K. L., Pott, J. -U., Esser, J., Pietrzyński, G., Nuñez, F. Pozo, Gianniotis, N., Blex, J., Lisow, T., Chini, R., Polsterer, K. L., Pott, J. -U., Esser, J., and Pietrzyński, G.

Abstract

We present the results of a two year optical continuum photometric reverberation mapping campaign carried out on the nucleus of the Seyfert-1 galaxy Mrk509. Specially designed narrow-band filters were used in order to mitigate the line and pseudo-continuum contamination of the signal from the broad line region, while allowing for high-accuracy flux-calibration over a large field of view. We obtained light curves with a sub-day time sampling and typical flux uncertainties of $1\%$. The high photometric precision allowed us to measure inter-band continuum time delays of up to $\sim 2$ days across the optical range. The time delays are consistent with the relation $\tau \propto \lambda^{4/3}$ predicted for an optically thick and geometrically thin accretion disk model. The size of the disk is, however, a factor of 1.8 larger than predictions based on the standard thin-disk theory. We argue that, for the particular case of Mrk509, a larger black hole mass due to the unknown geometry scaling factor can reconcile the difference between the observations and theory., Comment: 20 pages, 11 figures, published on Monthly Notices of the Royal Astronomical Society

Published
2019
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20. The first spectroscopic dust reverberation programme on active galactic nuclei: the torus in NGC 5548

Author

Landt, H., Ward, M. J., Kynoch, D., Packham, C., Ferland, G. J., Lawrence, A., Pott, J. -U., Esser, J., Horne, K., Starkey, D. A., Malhotra, D., Fausnaugh, M. M., Peterson, B. M., Wilman, R. J., Riffel, R. A., Storchi-Bergmann, T., Barth, A. J., Villforth, C., Winkler, H., Landt, H., Ward, M. J., Kynoch, D., Packham, C., Ferland, G. J., Lawrence, A., Pott, J. -U., Esser, J., Horne, K., Starkey, D. A., Malhotra, D., Fausnaugh, M. M., Peterson, B. M., Wilman, R. J., Riffel, R. A., Storchi-Bergmann, T., Barth, A. J., Villforth, C., and Winkler, H.

Abstract

We have recently initiated the first spectroscopic dust reverberation programme on active galactic nuclei (AGN) in the near-infrared. Spectroscopy enables measurement of dust properties, such as flux, temperature and covering factor, with higher precision than photometry. In particular, it enables measurement of both luminosity-based dust radii and dust response times. Here we report results from a one-year campaign on NGC 5548. The hot dust responds to changes in the irradiating flux with a lag time of ~70 light-days, similar to what was previously found in photometric reverberation campaigns. The mean and rms spectra are similar, implying that the same dust component dominates both the emission and the variations. The dust lag time is consistent with the luminosity-based dust radius only if we assume a wavelength-independent dust emissivity-law, i.e. a blackbody, which is appropriate for grains of large sizes (of a few microns). For such grains the dust temperature is ~1450 K. Therefore, silicate grains have most likely evaporated and carbon is the main chemical component. But the hot dust is not close to its sublimation temperature, contrary to popular belief. This is further supported by our observation of temperature variations largely consistent with a heating/cooling process. Therefore, the inner dust-free region is enlarged and the dusty torus rather a "dusty wall", whose inner radius is expected to be luminosity-invariant. The dust-destruction mechanism that enlarges the dust-free region seems to partly affect also the dusty region. We observe a cyclical decrease in dust mass with implied dust reformation times of ~5-6 months., Comment: 22 pages, 13 figures, accepted for publication in MNRAS

Published
2019
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21. Optical continuum photometric reverberation mapping of the Seyfert-1 galaxy Mrk509

Author

Nuñez, F. Pozo, Gianniotis, N., Blex, J., Lisow, T., Chini, R., Polsterer, K. L., Pott, J. -U., Esser, J., Pietrzyński, G., Nuñez, F. Pozo, Gianniotis, N., Blex, J., Lisow, T., Chini, R., Polsterer, K. L., Pott, J. -U., Esser, J., and Pietrzyński, G.

Abstract

We present the results of a two year optical continuum photometric reverberation mapping campaign carried out on the nucleus of the Seyfert-1 galaxy Mrk509. Specially designed narrow-band filters were used in order to mitigate the line and pseudo-continuum contamination of the signal from the broad line region, while allowing for high-accuracy flux-calibration over a large field of view. We obtained light curves with a sub-day time sampling and typical flux uncertainties of $1\%$. The high photometric precision allowed us to measure inter-band continuum time delays of up to $\sim 2$ days across the optical range. The time delays are consistent with the relation $\tau \propto \lambda^{4/3}$ predicted for an optically thick and geometrically thin accretion disk model. The size of the disk is, however, a factor of 1.8 larger than predictions based on the standard thin-disk theory. We argue that, for the particular case of Mrk509, a larger black hole mass due to the unknown geometry scaling factor can reconcile the difference between the observations and theory., Comment: 20 pages, 11 figures, published on Monthly Notices of the Royal Astronomical Society

Published
2019
Full Text
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22. The first spectroscopic dust reverberation programme on active galactic nuclei: the torus in NGC 5548

Author

Landt, H., Ward, M. J., Kynoch, D., Packham, C., Ferland, G. J., Lawrence, A., Pott, J. -U., Esser, J., Horne, K., Starkey, D. A., Malhotra, D., Fausnaugh, M. M., Peterson, B. M., Wilman, R. J., Riffel, R. A., Storchi-Bergmann, T., Barth, A. J., Villforth, C., Winkler, H., Landt, H., Ward, M. J., Kynoch, D., Packham, C., Ferland, G. J., Lawrence, A., Pott, J. -U., Esser, J., Horne, K., Starkey, D. A., Malhotra, D., Fausnaugh, M. M., Peterson, B. M., Wilman, R. J., Riffel, R. A., Storchi-Bergmann, T., Barth, A. J., Villforth, C., and Winkler, H.

Abstract

We have recently initiated the first spectroscopic dust reverberation programme on active galactic nuclei (AGN) in the near-infrared. Spectroscopy enables measurement of dust properties, such as flux, temperature and covering factor, with higher precision than photometry. In particular, it enables measurement of both luminosity-based dust radii and dust response times. Here we report results from a one-year campaign on NGC 5548. The hot dust responds to changes in the irradiating flux with a lag time of ~70 light-days, similar to what was previously found in photometric reverberation campaigns. The mean and rms spectra are similar, implying that the same dust component dominates both the emission and the variations. The dust lag time is consistent with the luminosity-based dust radius only if we assume a wavelength-independent dust emissivity-law, i.e. a blackbody, which is appropriate for grains of large sizes (of a few microns). For such grains the dust temperature is ~1450 K. Therefore, silicate grains have most likely evaporated and carbon is the main chemical component. But the hot dust is not close to its sublimation temperature, contrary to popular belief. This is further supported by our observation of temperature variations largely consistent with a heating/cooling process. Therefore, the inner dust-free region is enlarged and the dusty torus rather a "dusty wall", whose inner radius is expected to be luminosity-invariant. The dust-destruction mechanism that enlarges the dust-free region seems to partly affect also the dusty region. We observe a cyclical decrease in dust mass with implied dust reformation times of ~5-6 months., Comment: 22 pages, 13 figures, accepted for publication in MNRAS

Published
2019
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23. Analyzing temporal variations of AGN emission line profiles in the context of (dusty) cloud structure formation in the broad line region

Author

Esser, J., Pott, J. -U., Landt, H., Vacca, W. D., Esser, J., Pott, J. -U., Landt, H., and Vacca, W. D.

Abstract

The formation processes and the exact appearance of the dust torus and broad line region (BLR) of active galactic nuclei (AGN) are under debate. Theoretical studies show a possible connection between the dust torus and BLR through a common origin in the accretion disk. However observationally the dust torus and BLR are typically studied separately. NGC~4151 is possibly one of the best suited Seyfert~1 galaxies for simultaneous examinations because of its high number of both photometric and spectroscopic observations in the past. Here we compare changes of the dust radius to shape variations of broad emission lines (BEL). While the radius of the dust torus decreased by almost a factor of two from 2004 to 2006 shape variations can be seen in the red wing of BELs of NGC~4151. These simultaneous changes are discussed in a dust and BEL formation scheme. We also use the BEL shape variations to assess possible cloud distributions, especially in azimuthal direction, which could be responsible for the observed variations. Our findings can best be explained in the framework of a dust inflated accretion disk. The changes in the BELs suggest that this dusty cloud formation does not happen continuously, and over the whole accretion disk, but on the contrary in spatially confined areas over rather short amount of times. We derive limits to the azimuthal extension of the observed localized BEL flux enhancement event., Comment: accepted for publication in A&A

Published
2018
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30. Analyzing temporal variations of AGN emission line profiles in the context of (dusty) cloud structure formation in the broad line region

Author

Esser, J., Pott, J. -U., Landt, H., Vacca, W. D., Esser, J., Pott, J. -U., Landt, H., and Vacca, W. D.

Abstract

The formation processes and the exact appearance of the dust torus and broad line region (BLR) of active galactic nuclei (AGN) are under debate. Theoretical studies show a possible connection between the dust torus and BLR through a common origin in the accretion disk. However observationally the dust torus and BLR are typically studied separately. NGC~4151 is possibly one of the best suited Seyfert~1 galaxies for simultaneous examinations because of its high number of both photometric and spectroscopic observations in the past. Here we compare changes of the dust radius to shape variations of broad emission lines (BEL). While the radius of the dust torus decreased by almost a factor of two from 2004 to 2006 shape variations can be seen in the red wing of BELs of NGC~4151. These simultaneous changes are discussed in a dust and BEL formation scheme. We also use the BEL shape variations to assess possible cloud distributions, especially in azimuthal direction, which could be responsible for the observed variations. Our findings can best be explained in the framework of a dust inflated accretion disk. The changes in the BELs suggest that this dusty cloud formation does not happen continuously, and over the whole accretion disk, but on the contrary in spatially confined areas over rather short amount of times. We derive limits to the azimuthal extension of the observed localized BEL flux enhancement event., Comment: accepted for publication in A&A

Published
2018
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31. Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry

Author

Baudo, F, Collins, P, Huth Kühne, A, Lévesque, H, Marco, P, Nemes, L, Pellegrini, F, Tengborn, L, Knoebl, P, Aspoeck, G, Heistinger, M, Knöbl, P, Makipernaa, A, André, H, Aouba, A, Bellucci, S, Beurrier, P, Borg, J, Darnige, L, Devignes, J, D'Oiron, R, Gautier, P, Gay, V, Girault, S, Gruel, Y, Guerin, V, Hézard, N, Khellaf, M, Koenig, M, Lifermann, F, Marlu, R, Ninet, J, Peynet, J, Quéméneur, T, Rothschild, C, Schleinitz, N, Sigaud, M, Trouillier, S, Voisin, S, Giebl, A, Holstein, K, Loreth, R, Steigerwald, U, Tiede, A, Theodossiades, G, Radvanyi, G, Schlammadinger, A, Barillari, G, Pasca, S, Caimi, T, Contino, L, D'Angelo Armando, C, Fattorini, A, Di Minno, G, Cerbone, A, Di Minno, D, D'Incà, M, Falanga, A, Maggioni, A, Lerede, T, Franchini, M, Gaidano, G, De Paoli, L, Gamba, G, Ghirardi, R, Girotto, M, Tasca, D, Grandone, E, Tiscia, G, Imberti, D, Iorio, A, Landolfi, R, Di Gennaro, L, Novarese, L, Mariani, G, Lapecorella, M, Marietta, M, Pedrazzi, P, Mazzucconi, M, Santoro, C, Morfini, M, Linari, S, Moratelli, S, Paolini, R, Piseddu, G, Poggio, R, Pogliani, E, Carpenedo, M, Remiddi, C, Santagostino, E, Mancuso, M, Santoro, R, Papaleo, G, Schinco, P, Borchiellini, A, Valeri, F, Scortechini, A, Siragusa, S, Sottilotta, G, Squizzato, A, Tagariello, G, Sartori, R, Tagliaferri, A, Di Perna, C, Rivolta, G, Testa, S, Paoletti, O, Toschi, V, Zanon, E, Brandolin, B, Hamulyák, K, Kamphuisen, P, Laros van Gorkom, B, Leebeek, F, Marten, N, Novakova, I, Schutgens, R, van der Linden, P, van Esser, J, van der Meer, J, Ypma, P, Campos, M, Aguilar, C, Altisent, C, Bermejo, N, Del Campo, R, Ferreiro Argüelles, M, González Boullosa, R, Gutiérrez Pimentel, M, Jiménez Yuste, V, Jose Felix, L, Mingot, M, Perez Garrido, R, Perez Gonzale, N, Prieto Garcia, M, Rodriguez Huerta, A, Sedano, C, Tolosa Munoz, A, Baghaei, F, Boehlen, F, Korte, W, Chowdary, P, Evans, G, Pavord, S, Rangarajan, S, Wilde, J, Aspoeck G, Heistinger M, Knöbl P, Makipernaa A, André H, Aouba A, Bellucci S, Beurrier P, Borg JY, Darnige L, Devignes J, d'Oiron R, Gautier P, Gay V, Girault S, Gruel Y, Guerin V, Hézard N, Khellaf M, Koenig M, Lévesque H, Lifermann F, Marlu R, Ninet J, Peynet J, Quéméneur T, Rothschild C, Schleinitz N, Sigaud M, Trouillier S, Voisin S, Giebl A, Holstein K, Huth Kühne A, Loreth RM, Steigerwald U, Tiede A, Theodossiades G, Nemes L, Radvanyi G, Schlammadinger A, Barillari G, Pasca S, Baudo F, Caimi T, Contino L, D'Angelo Armando CL, Fattorini A, Di Minno G, Cerbone AM, Di Minno D, D'incà M, Falanga A, Maggioni A, Lerede T, Franchini M, Gaidano G, De Paoli L, Gamba G, Ghirardi R, Girotto M, Tasca D, Grandone E, Tiscia G, Imberti D, Iorio A, Landolfi R, Di Gennaro L, Novarese L, Mariani G, Lapecorella M, Marietta M, Pedrazzi P, Mazzucconi MG, Santoro C, Morfini M, Linari S, Moratelli S, Paolini R, Piseddu G, Poggio R, POGLIANI, ENRICO MARIA, Carpenedo M, Remiddi C, Santagostino E, Mancuso ME, Santoro R, Papaleo G, Schinco P, Borchiellini A, Valeri F, Scortechini AR, Siragusa S, Sottilotta G, Squizzato A, Tagariello G, Sartori R, Tagliaferri AR, Di Perna C, Rivolta GF, Testa S, Paoletti O, Toschi V, Zanon E, Brandolin B, Hamulyák K, Kamphuisen P, Laros van Gorkom B, Leebeek FW, Marten N, Novakova I, Schutgens R, van der Linden PW, van Esser J, van der Meer J, Ypma P, Campos M, Aguilar C, Altisent C, Bermejo N, Del Campo R, Ferreiro Argüelles M, González Boullosa R, Gutiérrez Pimentel MJ, Jiménez Yuste V, Jose Felix L, Marco P, Mingot ME, Perez Garrido R, Perez Gonzale NZ, Prieto Garcia M, Rodriguez Huerta AM, Sedano C, Tolosa Munoz A, Baghaei F, Tengborn L, Boehlen F, Korte W, Chowdary P, Collins P, Evans G, Pavord S, Rangarajan S, Wilde J., Baudo, F, Collins, P, Huth Kühne, A, Lévesque, H, Marco, P, Nemes, L, Pellegrini, F, Tengborn, L, Knoebl, P, Aspoeck, G, Heistinger, M, Knöbl, P, Makipernaa, A, André, H, Aouba, A, Bellucci, S, Beurrier, P, Borg, J, Darnige, L, Devignes, J, D'Oiron, R, Gautier, P, Gay, V, Girault, S, Gruel, Y, Guerin, V, Hézard, N, Khellaf, M, Koenig, M, Lifermann, F, Marlu, R, Ninet, J, Peynet, J, Quéméneur, T, Rothschild, C, Schleinitz, N, Sigaud, M, Trouillier, S, Voisin, S, Giebl, A, Holstein, K, Loreth, R, Steigerwald, U, Tiede, A, Theodossiades, G, Radvanyi, G, Schlammadinger, A, Barillari, G, Pasca, S, Caimi, T, Contino, L, D'Angelo Armando, C, Fattorini, A, Di Minno, G, Cerbone, A, Di Minno, D, D'Incà, M, Falanga, A, Maggioni, A, Lerede, T, Franchini, M, Gaidano, G, De Paoli, L, Gamba, G, Ghirardi, R, Girotto, M, Tasca, D, Grandone, E, Tiscia, G, Imberti, D, Iorio, A, Landolfi, R, Di Gennaro, L, Novarese, L, Mariani, G, Lapecorella, M, Marietta, M, Pedrazzi, P, Mazzucconi, M, Santoro, C, Morfini, M, Linari, S, Moratelli, S, Paolini, R, Piseddu, G, Poggio, R, Pogliani, E, Carpenedo, M, Remiddi, C, Santagostino, E, Mancuso, M, Santoro, R, Papaleo, G, Schinco, P, Borchiellini, A, Valeri, F, Scortechini, A, Siragusa, S, Sottilotta, G, Squizzato, A, Tagariello, G, Sartori, R, Tagliaferri, A, Di Perna, C, Rivolta, G, Testa, S, Paoletti, O, Toschi, V, Zanon, E, Brandolin, B, Hamulyák, K, Kamphuisen, P, Laros van Gorkom, B, Leebeek, F, Marten, N, Novakova, I, Schutgens, R, van der Linden, P, van Esser, J, van der Meer, J, Ypma, P, Campos, M, Aguilar, C, Altisent, C, Bermejo, N, Del Campo, R, Ferreiro Argüelles, M, González Boullosa, R, Gutiérrez Pimentel, M, Jiménez Yuste, V, Jose Felix, L, Mingot, M, Perez Garrido, R, Perez Gonzale, N, Prieto Garcia, M, Rodriguez Huerta, A, Sedano, C, Tolosa Munoz, A, Baghaei, F, Boehlen, F, Korte, W, Chowdary, P, Evans, G, Pavord, S, Rangarajan, S, Wilde, J, Aspoeck G, Heistinger M, Knöbl P, Makipernaa A, André H, Aouba A, Bellucci S, Beurrier P, Borg JY, Darnige L, Devignes J, d'Oiron R, Gautier P, Gay V, Girault S, Gruel Y, Guerin V, Hézard N, Khellaf M, Koenig M, Lévesque H, Lifermann F, Marlu R, Ninet J, Peynet J, Quéméneur T, Rothschild C, Schleinitz N, Sigaud M, Trouillier S, Voisin S, Giebl A, Holstein K, Huth Kühne A, Loreth RM, Steigerwald U, Tiede A, Theodossiades G, Nemes L, Radvanyi G, Schlammadinger A, Barillari G, Pasca S, Baudo F, Caimi T, Contino L, D'Angelo Armando CL, Fattorini A, Di Minno G, Cerbone AM, Di Minno D, D'incà M, Falanga A, Maggioni A, Lerede T, Franchini M, Gaidano G, De Paoli L, Gamba G, Ghirardi R, Girotto M, Tasca D, Grandone E, Tiscia G, Imberti D, Iorio A, Landolfi R, Di Gennaro L, Novarese L, Mariani G, Lapecorella M, Marietta M, Pedrazzi P, Mazzucconi MG, Santoro C, Morfini M, Linari S, Moratelli S, Paolini R, Piseddu G, Poggio R, POGLIANI, ENRICO MARIA, Carpenedo M, Remiddi C, Santagostino E, Mancuso ME, Santoro R, Papaleo G, Schinco P, Borchiellini A, Valeri F, Scortechini AR, Siragusa S, Sottilotta G, Squizzato A, Tagariello G, Sartori R, Tagliaferri AR, Di Perna C, Rivolta GF, Testa S, Paoletti O, Toschi V, Zanon E, Brandolin B, Hamulyák K, Kamphuisen P, Laros van Gorkom B, Leebeek FW, Marten N, Novakova I, Schutgens R, van der Linden PW, van Esser J, van der Meer J, Ypma P, Campos M, Aguilar C, Altisent C, Bermejo N, Del Campo R, Ferreiro Argüelles M, González Boullosa R, Gutiérrez Pimentel MJ, Jiménez Yuste V, Jose Felix L, Marco P, Mingot ME, Perez Garrido R, Perez Gonzale NZ, Prieto Garcia M, Rodriguez Huerta AM, Sedano C, Tolosa Munoz A, Baghaei F, Tengborn L, Boehlen F, Korte W, Chowdary P, Collins P, Evans G, Pavord S, Rangarajan S, and Wilde J.

Abstract

Acquired hemophilia A is a rare bleeding disorder caused by autoantibodies to coagulation FVIII. Bleeding episodes at presentation are spontaneous and severe in most cases. Optimal hemostatic therapy is controversial, and available data are from observational and retrospective studies only. The EACH2 registry, a multicenter, pan-European, Web-based database, reports current patient management. The aim was to assess the control of first bleeding episodes treated with a bypassing agent (rFVIIa or aPCC), FVIII, or DDAVP among 501 registered patients. Of 482 patients with one or more bleeding episodes, 144 (30%) received no treatment for bleeding; 31 were treated with symptomatic therapy only. Among 307 patients treated with a first-line hemostatic agent, 174 (56.7%) received rFVIIa, 63 (20.5%) aPCC, 56 (18.2%) FVIII, and 14 (4.6%) DDAVP. Bleeding was controlled in 269 of 338 (79.6%) patients treated with a first-line hemostatic agent or ancillary therapy alone. Propensity score matching was applied to allow unbiased comparison between treatment groups. Bleeding control was significantly higher in patients treated with bypassing agents versus FVIII/DDAVP (93.3% vs 68.3%; P = .003). Bleeding control was similar between rFVIIa and aPCC (93.0%; P = 1). Thrombotic events were reported in 3.6% of treated patients with a similar incidence between rFVIIa (2.9%) and aPCC (4.8%).

Published
2012

32. Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2)

Author

Knoebl, P, Marco, P, Baudo, F, Collins, P, Huth Kühne, A, Nemes, L, Pellegrini, F, Tengborn, L, Lévesque, H, Aspoeck, G, Heistinger, M, Knöbl, P, Makipernaa, A, André, H, Aouba, A, Bellucci, S, Beurrier, P, Borg, J, Darnige, L, Devignes, J, D'Oiron, R, Gautier, P, Gay, V, Girault, S, Gruel, Y, Guerin, V, Hézard, N, Khellaf, M, Koenig, M, Lifermann, F, Marlu, R, Peynet, J, Quéméneur, T, Rothschild, C, Schleinitz, N, Sigaud, M, Trouillier, S, Voisin, S, Giebl, A, Holstein, K, Loreth, R, Steigerwald, U, Tiede, A, Theodossiades, G, Radvanyi, G, Schlammadinger, A, Barillari, G, Pasca, S, Caimi, T, Contino, L, Di Minno, G, Cerbone, A, Di Minno, D, D'Incà, M, Falanga, A, Maggioni, A, Lerede, T, Franchini, M, Gaidano, G, De Paoli, L, Gamba, G, Ghirardi, R, Girotto, M, Tasca, D, Grandone, E, Tiscia, G, Imberti, D, Iorio, A, Landolfi, R, Di Gennaro, L, Novarese, L, Mariani, G, Lapecorella, M, Marietta, M, Pedrazzi, P, Mazzucconi, M, Santoro, C, Morfini, M, Linari, S, Moratelli, S, Paolini, R, Piseddu, G, Poggio, R, Pogliani, E, Carpenedo, M, Remiddi, C, Santagostino, E, Santoro, R, Papaleo, G, Schinco, P, Borchiellini, A, Scortechini, A, Siragusa, S, Sottilotta, G, Squizzato, A, Sartori, R, Tagariello, G, Tagliaferri, A, Di Perna, C, Rivolta, G, Testa, S, Paoletti, O, Toschi, V, Zanon, E, Brandolini, B, Hamulyák, K, Kamphuisen, P, Laros van Gorkom, B, Leebeek, F, Marten, N, Novakova, I, Schutgens, R, van der Linden, P, van Esser, J, van der Meer, J, Ypma, P, Campos, M, Aguilar, C, Altisent, C, Bermejo, N, Del Campo, R, Ferreiro Argüelles, M, González Boullosa, R, Gutiérrez Pimentel, M, Jiménez Yuste, V, Jose Felix, L, Pascual, M, Mingot, M, Perez Garrido, R, Perez Gonzale, N, Prieto Garcia, M, Rodriguez Huerta, A, Sedano, C, Tolosa Munoz, A, Baghaei, F, Boehlen, F, Korte, W, Chowdary, P, Evans, G, Pavord, S, Rangarajan, S, Wilde, J, Aspoeck G, Heistinger M, Knöbl P, Makipernaa A, André H, Aouba A, Bellucci S, Beurrier P, Borg JY, Darnige L, Devignes J, D'Oiron R, Gautier P, Gay V, Girault S, Gruel Y, Guerin V, Hézard N, Khellaf M, Koenig M, Lévesque H, Lifermann F, Marlu R, Peynet J, Quéméneur T, Rothschild C, Schleinitz N, Sigaud M, Trouillier S, Voisin S, Giebl A, Holstein K, Huth Kühne A, Loreth RM, Steigerwald U, Tiede A, Theodossiades G, Nemes L, Radvanyi G, Schlammadinger A, Barillari G, Pasca S, Baudo F, Caimi T, Contino L, Di Minno G, Cerbone AM, Di Minno D, D'incà M, Falanga A, Maggioni A, Lerede T, Franchini M, Gaidano G, De Paoli L, Gamba G, Ghirardi R, Girotto M, Tasca D, Grandone E, Tiscia G, Imberti D, Iorio A, Landolfi R, Di Gennaro L, Novarese L, Mariani G, Lapecorella M, Marietta M, Pedrazzi P, Mazzucconi MG, Santoro C, Morfini M, Linari S, Moratelli S, Paolini R, Piseddu G, Poggio R, POGLIANI, ENRICO MARIA, Carpenedo M, Remiddi C, Santagostino E, Santoro R, Papaleo G, Schinco P, Borchiellini A, Scortechini AR, Siragusa S, Sottilotta G, Squizzato A, Sartori R, Tagariello G, Tagliaferri AR, Di Perna C, Rivolta GF, Testa S, Paoletti O, Toschi V, Zanon E, Brandolini B, Hamulyák K, Kamphuisen P, Laros van Gorkom B, Leebeek FW, Marten N, Novakova I, Schutgens R, van der Linden PW, van Esser J, van der Meer J, Ypma P, Campos M, Aguilar C, Altisent C, Bermejo N, Del Campo R, Ferreiro Argüelles M, González Boullosa R, Gutiérrez Pimentel MJ, Jiménez Yuste V, Jose Felix L, Pascual M, Mingot ME, Perez Garrido R, Perez Gonzale Nz, Prieto Garcia M, Rodriguez Huerta AM, Sedano C, Tolosa Munoz A, Baghaei F, Tengborn L, Boehlen F, Korte W, Chowdary P, Collins P, Evans G, Pavord S, Rangarajan S, Wilde J., Knoebl, P, Marco, P, Baudo, F, Collins, P, Huth Kühne, A, Nemes, L, Pellegrini, F, Tengborn, L, Lévesque, H, Aspoeck, G, Heistinger, M, Knöbl, P, Makipernaa, A, André, H, Aouba, A, Bellucci, S, Beurrier, P, Borg, J, Darnige, L, Devignes, J, D'Oiron, R, Gautier, P, Gay, V, Girault, S, Gruel, Y, Guerin, V, Hézard, N, Khellaf, M, Koenig, M, Lifermann, F, Marlu, R, Peynet, J, Quéméneur, T, Rothschild, C, Schleinitz, N, Sigaud, M, Trouillier, S, Voisin, S, Giebl, A, Holstein, K, Loreth, R, Steigerwald, U, Tiede, A, Theodossiades, G, Radvanyi, G, Schlammadinger, A, Barillari, G, Pasca, S, Caimi, T, Contino, L, Di Minno, G, Cerbone, A, Di Minno, D, D'Incà, M, Falanga, A, Maggioni, A, Lerede, T, Franchini, M, Gaidano, G, De Paoli, L, Gamba, G, Ghirardi, R, Girotto, M, Tasca, D, Grandone, E, Tiscia, G, Imberti, D, Iorio, A, Landolfi, R, Di Gennaro, L, Novarese, L, Mariani, G, Lapecorella, M, Marietta, M, Pedrazzi, P, Mazzucconi, M, Santoro, C, Morfini, M, Linari, S, Moratelli, S, Paolini, R, Piseddu, G, Poggio, R, Pogliani, E, Carpenedo, M, Remiddi, C, Santagostino, E, Santoro, R, Papaleo, G, Schinco, P, Borchiellini, A, Scortechini, A, Siragusa, S, Sottilotta, G, Squizzato, A, Sartori, R, Tagariello, G, Tagliaferri, A, Di Perna, C, Rivolta, G, Testa, S, Paoletti, O, Toschi, V, Zanon, E, Brandolini, B, Hamulyák, K, Kamphuisen, P, Laros van Gorkom, B, Leebeek, F, Marten, N, Novakova, I, Schutgens, R, van der Linden, P, van Esser, J, van der Meer, J, Ypma, P, Campos, M, Aguilar, C, Altisent, C, Bermejo, N, Del Campo, R, Ferreiro Argüelles, M, González Boullosa, R, Gutiérrez Pimentel, M, Jiménez Yuste, V, Jose Felix, L, Pascual, M, Mingot, M, Perez Garrido, R, Perez Gonzale, N, Prieto Garcia, M, Rodriguez Huerta, A, Sedano, C, Tolosa Munoz, A, Baghaei, F, Boehlen, F, Korte, W, Chowdary, P, Evans, G, Pavord, S, Rangarajan, S, Wilde, J, Aspoeck G, Heistinger M, Knöbl P, Makipernaa A, André H, Aouba A, Bellucci S, Beurrier P, Borg JY, Darnige L, Devignes J, D'Oiron R, Gautier P, Gay V, Girault S, Gruel Y, Guerin V, Hézard N, Khellaf M, Koenig M, Lévesque H, Lifermann F, Marlu R, Peynet J, Quéméneur T, Rothschild C, Schleinitz N, Sigaud M, Trouillier S, Voisin S, Giebl A, Holstein K, Huth Kühne A, Loreth RM, Steigerwald U, Tiede A, Theodossiades G, Nemes L, Radvanyi G, Schlammadinger A, Barillari G, Pasca S, Baudo F, Caimi T, Contino L, Di Minno G, Cerbone AM, Di Minno D, D'incà M, Falanga A, Maggioni A, Lerede T, Franchini M, Gaidano G, De Paoli L, Gamba G, Ghirardi R, Girotto M, Tasca D, Grandone E, Tiscia G, Imberti D, Iorio A, Landolfi R, Di Gennaro L, Novarese L, Mariani G, Lapecorella M, Marietta M, Pedrazzi P, Mazzucconi MG, Santoro C, Morfini M, Linari S, Moratelli S, Paolini R, Piseddu G, Poggio R, POGLIANI, ENRICO MARIA, Carpenedo M, Remiddi C, Santagostino E, Santoro R, Papaleo G, Schinco P, Borchiellini A, Scortechini AR, Siragusa S, Sottilotta G, Squizzato A, Sartori R, Tagariello G, Tagliaferri AR, Di Perna C, Rivolta GF, Testa S, Paoletti O, Toschi V, Zanon E, Brandolini B, Hamulyák K, Kamphuisen P, Laros van Gorkom B, Leebeek FW, Marten N, Novakova I, Schutgens R, van der Linden PW, van Esser J, van der Meer J, Ypma P, Campos M, Aguilar C, Altisent C, Bermejo N, Del Campo R, Ferreiro Argüelles M, González Boullosa R, Gutiérrez Pimentel MJ, Jiménez Yuste V, Jose Felix L, Pascual M, Mingot ME, Perez Garrido R, Perez Gonzale Nz, Prieto Garcia M, Rodriguez Huerta AM, Sedano C, Tolosa Munoz A, Baghaei F, Tengborn L, Boehlen F, Korte W, Chowdary P, Collins P, Evans G, Pavord S, Rangarajan S, and Wilde J.

Abstract

Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies against coagulation factor VIII and characterized by spontaneous hemorrhage in patients with no previous family or personal history of bleeding. Although data on several AHA cohorts have been collected, limited information is available on the optimal management of AHA.

Published
2012

33. Pregnancy-associated acquired haemophilia A: results from the European Acquired Haemophilia (EACH2) registry

Author

Tengborn, L, Baudo, F, Huth-Kühne, A, Knoebl, P, Lévesque, H, Marco, P, Pellegrini, F, Nemes, L, Collins, P, EACH2 registry, C, Aspoeck, G, Heistinger, M, Knöbl, P, Makipernaa, A, André, H, Aouba, A, Bellucci, S, Beurrier, P, Borg, J, Darnige, L, Devignes, J, D'Oiron, R, Gautier, P, Gay, V, Girault, S, Gruel, Y, Guerin, V, Hézard, N, Khellaf, M, Koenig, M, Lifermann, F, Marlu, R, Ninet, J, Peynet, J, Quéméneur, T, Rothschild, C, Schleinitz, N, Sigaud, M, Trouillier, S, Voisin, S, Giebl, A, Holstein, K, Loreth, R, Steigerwald, U, Tiede, A, Theodossiades, G, Radvanyi, G, Schlammadinger, A, Barillari, G, Pasca, S, Caimi, T, Contino, L, D'Angelo Armando, C, Fattorini, A, Di Minno, G, Cerbone, A, Di Minno, D, D'Incà, M, Falanga, A, Maggioni, A, Lerede, T, Franchini, M, Gaidano, G, De Paoli, L, Gamba, G, Ghirardi, R, Girotto, M, Tasca, D, Grandone, E, Tiscia, G, Imberti, D, Iorio, A, Landolfi, R, Di Gennaro, L, Novarese, L, Mariani, G, Lapecorella, M, Marietta, M, Pedrazzi, P, Mazzucconi, M, Santoro, C, Morfini, M, Linari, S, Moratelli, S, Paolini, R, Piseddu, G, Poggio, R, Pogliani, E, Carpenedo, M, Remiddi, C, Santagostino, E, Mancuso, M, Santoro, R, Papaleo, G, Schinco, P, Borchiellini, A, Valeri, F, Scortechini, A, Siragusa, S, Sottilotta, G, Squizzato, A, Tagariello, G, Sartori, R, Tagliaferri, A, Di Perna, C, Rivolta, G, Testa, S, Paoletti, O, Toschi, V, Zanon, E, Hamulyák, K, Kamphuisen, P, Laros-van Gorkom, B, Leebeek, F, Marten, N, Novakova, I, Schutgens, R, van der Linden, P, van Esser, J, van der Meer, J, Ypma, P, Campos, M, Aguilar, C, Altisent, C, Bermejo, N, Del Campo, R, Ferreiro Argüelles, M, González Boullosa, R, Gutiérrez Pimentel, M, Jiménez-Yuste, V, Jose-Felix, L, Mingot, M, Perez Garrido, R, Perez Gonzale, N, Prieto Garcia, M, Rodriguez-Huerta, A, Sedano, C, Tolosa Munoz, A, Baghaei, F, Boehlen, F, Korte, W, Chowdary, P, Evans, G, Pavord, S, Rangarajan, S, Wilde, J, Tengborn L, Baudo F, Huth-Kühne A, Knoebl P, Lévesque H, Marco P, Pellegrini F, Nemes L, Collins P, EACH2 registry contributors, Aspoeck G, Heistinger M, Knöbl P, Makipernaa A, André H, Aouba A, Bellucci S, Beurrier P, Borg JY, Darnige L, Devignes J, d'Oiron R, Gautier P, Gay V, Girault S, Gruel Y, Guerin V, Hézard N, Khellaf M, Koenig M, Lifermann F, Marlu R, Ninet J, Peynet J, Quéméneur T, Rothschild C, Schleinitz N, Sigaud M, Trouillier S, Voisin S, Giebl A, Holstein K, Loreth RM, Steigerwald U, Tiede A, Theodossiades G, Radvanyi G, Schlammadinger A, Barillari G, Pasca S, Caimi T, Contino L, D'Angelo Armando CL, Fattorini A, Di Minno G, Cerbone AM, Di Minno D, D'incà M, Falanga A, Maggioni A, Lerede T, Franchini M, Gaidano G, De Paoli L, Gamba G, Ghirardi R, Girotto M, Tasca D, Grandone E, Tiscia G, Imberti D, Iorio A, Landolfi R, Di Gennaro L, Novarese L, Mariani G, Lapecorella M, Marietta M, Pedrazzi P, Mazzucconi MG, Santoro C, Morfini M, Linari S, Moratelli S, Paolini R, Piseddu G, Poggio R, Pogliani E, Carpenedo M, Remiddi C, Santagostino E, Mancuso ME, Santoro R, Papaleo G, Schinco P, Borchiellini A, Valeri F, Scortechini AR, Siragusa S, Sottilotta G, Squizzato A, Tagariello G, Sartori R, Tagliaferri AR, Di Perna C, Rivolta GF, Testa S, Paoletti O, Toschi V, Zanon E, Hamulyák K, Kamphuisen P, Laros-van Gorkom B, Leebeek FW, Marten N, Novakova I, Schutgens R, van der Linden PW, van Esser J, van der Meer J, Ypma P, Campos M, Aguilar C, Altisent C, Bermejo N, Del Campo R, Ferreiro Argüelles M, González Boullosa R, Gutiérrez Pimentel MJ, Jiménez-Yuste V, Jose-Felix L, Mingot ME, Perez Garrido R, Perez Gonzale NZ, Prieto Garcia M, Rodriguez-Huerta AM, Sedano C, Tolosa Munoz A, Baghaei F, Boehlen F, Korte W, Chowdary P, Evans G, Pavord S, Rangarajan S, Wilde J, Tengborn, L, Baudo, F, Huth-Kühne, A, Knoebl, P, Lévesque, H, Marco, P, Pellegrini, F, Nemes, L, Collins, P, EACH2 registry, C, Aspoeck, G, Heistinger, M, Knöbl, P, Makipernaa, A, André, H, Aouba, A, Bellucci, S, Beurrier, P, Borg, J, Darnige, L, Devignes, J, D'Oiron, R, Gautier, P, Gay, V, Girault, S, Gruel, Y, Guerin, V, Hézard, N, Khellaf, M, Koenig, M, Lifermann, F, Marlu, R, Ninet, J, Peynet, J, Quéméneur, T, Rothschild, C, Schleinitz, N, Sigaud, M, Trouillier, S, Voisin, S, Giebl, A, Holstein, K, Loreth, R, Steigerwald, U, Tiede, A, Theodossiades, G, Radvanyi, G, Schlammadinger, A, Barillari, G, Pasca, S, Caimi, T, Contino, L, D'Angelo Armando, C, Fattorini, A, Di Minno, G, Cerbone, A, Di Minno, D, D'Incà, M, Falanga, A, Maggioni, A, Lerede, T, Franchini, M, Gaidano, G, De Paoli, L, Gamba, G, Ghirardi, R, Girotto, M, Tasca, D, Grandone, E, Tiscia, G, Imberti, D, Iorio, A, Landolfi, R, Di Gennaro, L, Novarese, L, Mariani, G, Lapecorella, M, Marietta, M, Pedrazzi, P, Mazzucconi, M, Santoro, C, Morfini, M, Linari, S, Moratelli, S, Paolini, R, Piseddu, G, Poggio, R, Pogliani, E, Carpenedo, M, Remiddi, C, Santagostino, E, Mancuso, M, Santoro, R, Papaleo, G, Schinco, P, Borchiellini, A, Valeri, F, Scortechini, A, Siragusa, S, Sottilotta, G, Squizzato, A, Tagariello, G, Sartori, R, Tagliaferri, A, Di Perna, C, Rivolta, G, Testa, S, Paoletti, O, Toschi, V, Zanon, E, Hamulyák, K, Kamphuisen, P, Laros-van Gorkom, B, Leebeek, F, Marten, N, Novakova, I, Schutgens, R, van der Linden, P, van Esser, J, van der Meer, J, Ypma, P, Campos, M, Aguilar, C, Altisent, C, Bermejo, N, Del Campo, R, Ferreiro Argüelles, M, González Boullosa, R, Gutiérrez Pimentel, M, Jiménez-Yuste, V, Jose-Felix, L, Mingot, M, Perez Garrido, R, Perez Gonzale, N, Prieto Garcia, M, Rodriguez-Huerta, A, Sedano, C, Tolosa Munoz, A, Baghaei, F, Boehlen, F, Korte, W, Chowdary, P, Evans, G, Pavord, S, Rangarajan, S, Wilde, J, Tengborn L, Baudo F, Huth-Kühne A, Knoebl P, Lévesque H, Marco P, Pellegrini F, Nemes L, Collins P, EACH2 registry contributors, Aspoeck G, Heistinger M, Knöbl P, Makipernaa A, André H, Aouba A, Bellucci S, Beurrier P, Borg JY, Darnige L, Devignes J, d'Oiron R, Gautier P, Gay V, Girault S, Gruel Y, Guerin V, Hézard N, Khellaf M, Koenig M, Lifermann F, Marlu R, Ninet J, Peynet J, Quéméneur T, Rothschild C, Schleinitz N, Sigaud M, Trouillier S, Voisin S, Giebl A, Holstein K, Loreth RM, Steigerwald U, Tiede A, Theodossiades G, Radvanyi G, Schlammadinger A, Barillari G, Pasca S, Caimi T, Contino L, D'Angelo Armando CL, Fattorini A, Di Minno G, Cerbone AM, Di Minno D, D'incà M, Falanga A, Maggioni A, Lerede T, Franchini M, Gaidano G, De Paoli L, Gamba G, Ghirardi R, Girotto M, Tasca D, Grandone E, Tiscia G, Imberti D, Iorio A, Landolfi R, Di Gennaro L, Novarese L, Mariani G, Lapecorella M, Marietta M, Pedrazzi P, Mazzucconi MG, Santoro C, Morfini M, Linari S, Moratelli S, Paolini R, Piseddu G, Poggio R, Pogliani E, Carpenedo M, Remiddi C, Santagostino E, Mancuso ME, Santoro R, Papaleo G, Schinco P, Borchiellini A, Valeri F, Scortechini AR, Siragusa S, Sottilotta G, Squizzato A, Tagariello G, Sartori R, Tagliaferri AR, Di Perna C, Rivolta GF, Testa S, Paoletti O, Toschi V, Zanon E, Hamulyák K, Kamphuisen P, Laros-van Gorkom B, Leebeek FW, Marten N, Novakova I, Schutgens R, van der Linden PW, van Esser J, van der Meer J, Ypma P, Campos M, Aguilar C, Altisent C, Bermejo N, Del Campo R, Ferreiro Argüelles M, González Boullosa R, Gutiérrez Pimentel MJ, Jiménez-Yuste V, Jose-Felix L, Mingot ME, Perez Garrido R, Perez Gonzale NZ, Prieto Garcia M, Rodriguez-Huerta AM, Sedano C, Tolosa Munoz A, Baghaei F, Boehlen F, Korte W, Chowdary P, Evans G, Pavord S, Rangarajan S, and Wilde J

Abstract

Objective The European Acquired Haemophilia registry (EACH2) collected data on the demographics, diagnosis, underlying disorders, bleeding characteristics, treatment, and outcome of women with acquired haemophilia A (AHA), a rare and often severe bleeding disorder caused by autoantibodies directed against coagulation factor VIII. Design Prospective, multi-centre, large-scale, pan-European registry. Setting A total of 117 haemophilia centres in 13 European countries. Population Pregnancy-associated AHA. Methods Data were reported using a web-based electronic case report form. Diagnosis was based on the presence of a prolonged activated partial thromboplastin time, reduced coagulation Factor VIII level and positive inhibitor assay. Main outcome measures Presenting characteristics, time to diagnosis, haemostatic treatment and outcome, immunosuppressive treatment and outcome. Results The EACH2 registry (n = 501) documented 42 (8.4%) cases of AHA associated with the peripartum period, a median Factor VIII level at diagnosis of 2.5 (range 0-25) IU/dl and inhibitor titre of 7.8 (range 0.7-348) BU/ml. Antepartum inhibitors were evident in eight women. Time to diagnosis of AHA after delivery was 89 (range 21-120) days. First-line haemostatic treatment was successful in 20/23 (87%) women treated. Bleeding episodes resolved in 17/18 (94%) women treated with a bypassing agent and 29/39 (74%) women achieved complete remission with first-line immunosuppressive treatment. Two babies experienced postnatal bleeding, suggesting transplacental transfer of the antibody. All women were alive at last follow-up. Conclusions Although rare, pregnancy-associated AHA may cause severe bleeding-related morbidity. Once diagnosed, women respond well to haemostatic treatment with bypassing agents and immunosuppression. Awareness of peripartum AHA requires improvement to facilitate rapid and appropriate management.

Published
2012

35. Evaluation of a retinal imaging laser eye wear

Author

Oeverhaus, MM, Dekowski, D, Stöhr, M, Holtemeyer, J, Schaperdoth-Gerlings, B, Esser, J, Eckstein, A, Oeverhaus, MM, Dekowski, D, Stöhr, M, Holtemeyer, J, Schaperdoth-Gerlings, B, Esser, J, and Eckstein, A

Published
2017

41. Evaluation of a retinal imaging laser eye wear

Author

Oeverhaus, MM, Dekowski, D, Stöhr, M, Holtemeyer, J, Schaperdoth-Gerlings, B, Esser, J, Eckstein, A, Oeverhaus, MM, Dekowski, D, Stöhr, M, Holtemeyer, J, Schaperdoth-Gerlings, B, Esser, J, and Eckstein, A

Published
2017

46. Properties of optically selected BL Lac candidates from the SDSS

Author

Kügler, S. D., Nilsson, K., Heidt, J., Esser, J., Schultz, T., Kügler, S. D., Nilsson, K., Heidt, J., Esser, J., and Schultz, T.

Abstract

\textbf{Context.} Deep optical surveys open the avenue for find large numbers of BL Lac objects that are hard to identify because they lack the unique properties classifying them as such. While radio or X-ray surveys typically reveal dozens of sources, recent compilations based on optical criteria alone have increased the number of BL Lac candidates considerably. However, these compilations are subject to biases and may contain a substantial number of contaminating sources. \textbf{Aims.} In this paper we extend our analysis of 182 optically selected BL Lac object candidates from the SDSS with respect to an earlier study. The main goal is to determine the number of bona fide BL Lac objects in this sample. \textbf{Methods.} We examine their variability characteristics, determine their broad-band radio-UV SEDs, and search for the presence of a host galaxy. In addition we present new optical spectra for 27 targets with improved S/N with respect to the SDSS spectra. \textbf{Results.} At least 59% of our targets have shown variability between SDSS DR2 and our observations by more than 0.1-0.27 mag de- pending on the telescope used. A host galaxy was detected in 36% of our targets. The host galaxy type and luminosities are consistent with earlier studies of BL Lac host galaxies. Simple fits to broad-band SEDS for 104 targets of our sample derived synchrotron peak frequencies between $13.5 \leq \mathrm{log}_{10}(\nu_{\mathrm{peak}}) \leq 16$ with a peak at $\mathrm{log}_{10} \sim 14.5$. Our new optical spectra do not reveal any new redshift for any of our objects. Thus the sample contains a large number of bona fide BL Lac objects and seems to contain a substantial fraction of intermediate-frequency peaked BL Lacs., Comment: Accepted for publication in A\&A

Published
2014
Full Text
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49. Properties of optically selected BL Lac candidates from the SDSS

Author

Kügler, S. D., Nilsson, K., Heidt, J., Esser, J., Schultz, T., Kügler, S. D., Nilsson, K., Heidt, J., Esser, J., and Schultz, T.

Abstract

\textbf{Context.} Deep optical surveys open the avenue for find large numbers of BL Lac objects that are hard to identify because they lack the unique properties classifying them as such. While radio or X-ray surveys typically reveal dozens of sources, recent compilations based on optical criteria alone have increased the number of BL Lac candidates considerably. However, these compilations are subject to biases and may contain a substantial number of contaminating sources. \textbf{Aims.} In this paper we extend our analysis of 182 optically selected BL Lac object candidates from the SDSS with respect to an earlier study. The main goal is to determine the number of bona fide BL Lac objects in this sample. \textbf{Methods.} We examine their variability characteristics, determine their broad-band radio-UV SEDs, and search for the presence of a host galaxy. In addition we present new optical spectra for 27 targets with improved S/N with respect to the SDSS spectra. \textbf{Results.} At least 59% of our targets have shown variability between SDSS DR2 and our observations by more than 0.1-0.27 mag de- pending on the telescope used. A host galaxy was detected in 36% of our targets. The host galaxy type and luminosities are consistent with earlier studies of BL Lac host galaxies. Simple fits to broad-band SEDS for 104 targets of our sample derived synchrotron peak frequencies between $13.5 \leq \mathrm{log}_{10}(\nu_{\mathrm{peak}}) \leq 16$ with a peak at $\mathrm{log}_{10} \sim 14.5$. Our new optical spectra do not reveal any new redshift for any of our objects. Thus the sample contains a large number of bona fide BL Lac objects and seems to contain a substantial fraction of intermediate-frequency peaked BL Lacs., Comment: Accepted for publication in A\&A

Published
2014
Full Text
View/download PDF

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