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Small sizes and indolent evolutionary dynamics challenge the potential role of P2RY8-CRLF2-harboring clones as main relapse-driving force in childhood ALL (2012)

Morak, M., Attarbaschi, A., Fischer, S., Nassimbeni, C., Grausenburger, R., Bastelberger, S., Krentz, S., Cario, G., Kasper, D., Schmitt, K., Russell, L. J., Potschger, U., Stanulla, M., Eckert, C., Mann, G., Haas, O. A., Panzer-Grumayer, R.

American Society of Hematology (ASH)

In: Blood

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Publication Date: 2012-12-21

Description: The P2RY8-CRLF2 fusion defines a particular relapse-prone subset of childhood acute lymphoblastic leukemia (ALL) in Italian Association of Pediatric Hematology and Oncology Berlin-Frankfurt-Münster (AIEOP-BFM) 2000 protocols. To investigate whether and to what extent different clone sizes influence disease and relapse development, we quantified the genomic P2RY8-CRLF2 fusion product and correlated it with the corresponding CRLF2 expression levels in patients enrolled in the BFM-ALL 2000 protocol in Austria. Of 268 cases without recurrent chromosomal translocations and high hyperdiploidy, representing approximately 50% of all cases, 67 (25%) were P2RY8-CRLF2 positive. The respective clone sizes were ≥ 20% in 27% and 〈 20% in 73% of them. The cumulative incidence of relapse of the entire fusion-positive group was clone size independent and significantly higher than that of the fusion-negative group (35% ± 8% vs 13% ± 3%, P = .008) and primarily confined to the non–high-risk group. Of 22 P2RY8-CRLF2 –positive diagnosis/relapse pairs, only 4/8 had the fusion-positive dominant clone conserved at relapse, whereas none of the original 14 fusion-positive small clones reappeared as the dominant relapse clone. We conclude that the majority of P2RY8-CRLF2 –positive clones are small at diagnosis and virtually never generate a dominant relapse clone. Our findings therefore suggest that P2RY8-CRLF2 –positive clones do not have the necessary proliferative or selective advantage to evolve into a disease-relevant relapse clone.

Keywords: Free Research Articles, Lymphoid Neoplasia, 〈a href="/content/by/section/Editorials"〉Editorials〈/a〉, Clinical Trials and Observations

Print ISSN: 0006-4971

Electronic ISSN: 1528-0020

Topics: Biology , Medicine

Published by American Society of Hematology (ASH)

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Feasibility of semiquantitative liver perfusion assessment by ferucarbotran bolus injection in double-contrast hepatic MRI (2012)

Michael A. Fischer, Olivio F. Donati, Caecilia S. Reiner, Roger Hunziker, Daniel Nanz, Andreas Boss

Wiley-Blackwell

In: Journal of Magnetic Resonance Imaging

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Publication Date: 2012-04-16

Description: Purpose: To evaluate the feasibility of semiquantitative measurement of liver perfusion from analysis of ferucarbotran induced signal-dynamics in double-contrast liver MR-imaging (DC-MRI). Materials and Methods: In total 31 patients (21 men; 58 ± 10 years) including 18 patients with biopsy proven liver cirrhosis prospectively underwent clinically indicated DC-MRI at 1.5 Tesla (T) with dynamic T2*-weighted gradient-echo imaging after ferucarbotran bolus injection. Breathing artefacts in tissue and input time curves were reduced by Savitzky-Golay-filtering and semiquantitative perfusion maps were calculated using a model free approach. Hepatic blood flow index (HBFI) and splenic blood flow index (SBFI) were determined by normalization of arbitrary perfusion values to the perfusion of the erector spinae muscle resulting in a semiquantitative perfusion measure. Results: In 30 of 31 patients the evaluated protocol could successfully be applied. Mean HBF was 7.7 ± 2.46 (range, 4.6–12.8) and mean SBF was 13.20 ± 2.57 (range, 8.5–17.8). A significantly lower total HBF was seen in patients with cirrhotic livers as compared to patients with noncirrhotic livers ( P 〈 0.05). In contrast, similar SBF was observed in cirrhotic and noncirrhotic patients ( P = 0.11). Conclusion: Capturing the signal dynamics during bolus injection of ferucarbotran in DC-MRI of the liver allows for semiquantitative assessment of hepatic perfusion that may be helpful for a more precise characterisation of liver cirrhosis and focal liver lesions. J. Magn. Reson. Imaging 2012;. © 2012 Wiley Periodicals, Inc.

Print ISSN: 1053-1807

Electronic ISSN: 1522-2586

Topics: Medicine

Published by Wiley-Blackwell

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catena-Poly[[(tetrahydrofuran-κO)potassium]-μ-(η5:η5)-2,3,4,5-tetramethyl-1-n-pentylcyclopentadienyl] (2013)

Rüdiger W. Seidel, Chelladurai Ganesamoorthy, Sinah Loerke, Manuela V. Winter, Christian Gemel, Roland A. Fischer

Wiley-Blackwell

In: Acta Crystallographica Section C

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Publication Date: 2013-05-10

Description: The title compound, [K(C 14 H 23 )(C 4 H 8 O)] n , comprises zigzag chains of alternating bridging 2,3,4,5-tetramethyl-1- n -pentylcyclopentadienyl ligands and potassium ions, with an ancillary tetrahydrofuran ligand in the coordination environment of potassium. The coordination polymer strands so formed extend by 2 1 screw symmetry in the b -axis direction. The chemically modified cyclopentadienyl ligand, with a tethered n -pentyl group, was synthesized from 2,3,4,5-tetramethylcyclopent-2-enone by a Grignard reaction.

Print ISSN: 0108-2701

Electronic ISSN: 1600-5759

Topics: Chemistry and Pharmacology , Geosciences , Physics

Published by Wiley-Blackwell on behalf of International Union of Crystallography (IUCr).

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Outcomes of transplantation using various hematopoietic cell sources in children with Hurler syndrome after myeloablative conditioning (2013)

Boelens, J. J., Aldenhoven, M., Purtill, D., Ruggeri, A., De; For, T., Wynn, R., Wraith, E., Cavazzana-Calvo, M., Rovelli, A., Fischer, A., Tolar, J., Prasad, V. K., Escolar, M., Gluckman, E., O'Meara, A., Orchard, P. J., Veys, P., Eapen, M., Kurtzberg, J., Rocha, V., on behalf of all participating centers from Eurocord, Inborn Errors Working Party of European Blood and Marrow Transplant group, Duke University, and the Centre for International Blood and Marrow Research

American Society of Hematology (ASH)

In: Blood

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Publication Date: 2013-05-10

Description: We report transplantation outcomes of 258 children with Hurler syndrome (HS) after a myeloablative conditioning regimen from 1995 to 2007. Median age at transplant was 16.7 months and median follow-up was 57 months. The cumulative incidence of neutrophil recovery at day 60 was 91%, acute graft-versus-host disease (GVHD) (grade II-IV) at day 100 was 25%, and chronic GVHD and 5 years was 16%. Overall survival and event-free survival (EFS) at 5 years were 74% and 63%, respectively. EFS after HLA-matched sibling donor (MSD) and 6/6 matched unrelated cord blood (CB) donor were similar at 81%, 66% after 10/10 HLA-matched unrelated donor (UD), and 68% after 5/6 matched CB donor. EFS was lower after transplantation in 4/6 matched unrelated CB (UCB) (57%; P = .031) and HLA-mismatched UD (41%; P = .007). Full-donor chimerism ( P = .039) and normal enzyme levels ( P = .007) were higher after CB transplantation (92% and 98%, respectively) compared with the other grafts sources (69% and 59%, respectively). In conclusion, results of allogeneic transplantation for HS are encouraging, with similar EFS rates after MSD, 6/6 matched UCB, 5/6 UCB, and 10/10 matched UD. The use of mismatched UD and 4/6 matched UCB was associated with lower EFS.

Keywords: Pediatric Hematology, Transplantation, Free Research Articles

Print ISSN: 0006-4971

Electronic ISSN: 1528-0020

Topics: Biology , Medicine

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Performance of a four-element Si drift detector for X-ray absorption fine-structure spectroscopy: resolution, maximum count rate, and dead-time correction with incorporation into the ATHENA data analysis software (2015)

J. C. Woicik, B. Ravel, D. A. Fischer, W. J. Newburgh

Wiley-Blackwell

In: Journal of Synchrotron Radiation [Synchrotron Radiation Online]

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Publication Date: 2015-12-08

Description: The performance of a four-element Si drift detector for energy-dispersive fluorescence-yield X-ray absorption fine-structure measurements is reported, operating at the National Institute of Standards and Technology beamline X23A2 at the National Synchrotron Light Source. The detector can acquire X-ray absorption fine-structure spectra with a throughput exceeding 4 × 10 5 counts per second per detector element (〉1.6 × 10 6 total counts per second summed over all four channels). At this count rate the resolution at 6 keV is approximately 220 eV, which adequately resolves the Mn K α and K β fluorescence lines. Accurate dead-time correction is demonstrated, and it has been incorporated into the ATHENA data analysis program. To maintain counting efficiency and high signal to background, it is suggested that the incoming count rate should not exceed ∼70% of the maximum throughput.

Print ISSN: 0909-0495

Electronic ISSN: 1600-5775

Topics: Geosciences , Physics

Published by Wiley-Blackwell on behalf of International Union of Crystallography (IUCr).

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Stem cell transplantation in severe congenital neutropenia: an analysis from the European Society for Blood and Marrow Transplantation (2015)

Fioredda, F., Iacobelli, S., van Biezen, A., Gaspar, B., Ancliff, P., Donadieu, J., Aljurf, M., Peters, C., Calvillo, M., Matthes-Martin, S., Morreale, G., van 't Veer-Tazelaar, N., de Wreede, L., Al Seraihy, A., Yesilipek, A., Fischer, A., Bierings, M., Ozturk, G., Smith, O., Veys, P., Ljungman, P., Peffault de Latour, R., Sanchez de Toledo Codina, J., Or, R., Ganser, A., Afanasyev, B., Wynn, R., Kalwak, K., Marsh, J., Dufour, C., on behalf of the Severe Aplastic Anemia the Inborn Error, and the Pediatric Disease Working Parties of the European Society for Blood and Bone Marrow Transplantation (EBMT) and Stem Cell Transplant for Immunodeficiencies in Europe (SCETIDE)

American Society of Hematology (ASH)

In: Blood

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Publication Date: 2015-10-16

Description: Allogeneic hematopoietic stem cell transplantation (HSCT) is the only curative treatment of severe congenital neutropenia (SCN), but data on outcome are scarce. We report on the outcome of 136 SCN patients who underwent HSCT between 1990 and 2012 in European and Middle East centers. The 3-year overall survival (OS) was 82%, and transplant-related mortality (TRM) was 17%. In multivariate analysis, transplants performed under the age of 10 years, in recent years, and from HLA-matched related or unrelated donors were associated with a significantly better OS. Frequency of graft failure was 10%. Cumulative incidence (day +90) of acute graft-versus-host disease (GVHD) grade 2-4 was 21%. In multivariate analysis, HLA-matched related donor and prophylaxis with cyclosporine A and methotrexate were associated with lower occurrence of acute GVHD. Cumulative incidence (1 year) of chronic GVHD was 20%. No secondary malignancies occurred after a median follow-up of 4.6 years. These data show that the outcome of HSCT for SCN from HLA-matched donors, performed in recent years, in patients younger than 10 years is acceptable. Nevertheless, given the TRM, a careful selection of HSCT candidates should be undertaken.

Keywords: Hematopoiesis and Stem Cells, Pediatric Hematology, Transplantation, Free Research Articles, Phagocytes, Granulocytes, and Myelopoiesis, CME article, Clinical Trials and Observations

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Electronic ISSN: 1528-0020

Topics: Biology , Medicine

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High-Pressure, High-Temperature Equations of State Using Nanofabricated Controlled-Geometry Ni/SiO2/Ni Double Hot-Plate Samples (2015)

Jeffrey S. Pigott, Derek A. Ditmer, Rebecca A. Fischer, Daniel M. Reaman, Rostislav Hrubiak, Yue Meng, Robert J. Davis, Wendy R. Panero

Wiley-Blackwell

In: Geophysical Research Letters

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Publication Date: 2015-11-26

Description: We have fabricated novel controlled-geometry samples for the laser-heated diamond anvil cell (LHDAC) in which a transparent oxide layer (SiO 2 ) is sandwiched between two laser-absorbing layers (Ni) in a single, cohesive sample. The samples were mass manufactured (〉10 4 samples) using a combination of physical vapor deposition, photolithography, and wet and plasma etching. The double hot-plate arrangement of the samples, coupled with the chemical and spatial homogeneity of the laser-absorbing layers, addresses problems of spatial temperature heterogeneities encountered in previous studies where simple mechanical mixtures of transparent and opaque materials were used. Here we report thermal equations of state (EOS) for nickel to 100 GPa and 3000 K and stishovite to 50 GPa and 2400 K obtained using the LHDAC and in situ synchrotron x-ray micro-diffraction. We discuss the inner core composition and the stagnation of subducted slabs in the mantle based on our refined thermal EOS.

Print ISSN: 0094-8276

Electronic ISSN: 1944-8007

Topics: Geosciences , Physics

Published by Wiley-Blackwell on behalf of American Geophysical Union (AGU).

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Five percent of healthy newborns have an ETV6-RUNX1 fusion as revealed by DNA-based GIPFEL screening (2018)

Schäfer, D., Olsen, M., Lähnemann, D., Stanulla, M., Slany, R., Schmiegelow, K., Borkhardt, A., Fischer, U.

American Society of Hematology (ASH)

In: Blood

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Publication Date: 2018-02-16

Keywords: Pediatric Hematology, Lymphoid Neoplasia

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Topics: Biology , Medicine

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Common climatic signal from glaciers in the European Alps over the last 50 years (2017)

C. Vincent, A. Fischer, C. Mayer, A. Bauder, S. P. Galos, M. Funk, E. Thibert, D. Six, L. Braun, M. Huss

Wiley-Blackwell

In: Geophysical Research Letters

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Publication Date: 2017-01-29

Description: Conventional glacier-wide mass balances are commonly used to study the effect of climate forcing on glacier melt. Unfortunately, the glacier-wide mass balances are also influenced by the glacier's dynamic response. Investigations on the effects of climate forcing on glaciers can be largely improved by analyzing point mass balances. Using a statistical model, we have found that 52% of the year-to-year deviations in the point mass balances of six glaciers distributed across the entire European Alps can be attributed to a common variability. Point mass balance changes reveal remarkable regional consistencies reaching 80% for glaciers less than 10 km apart. Compared to the steady-state conditions of the 1962-1982 period, the surface mass balance changes are -0.85 m w.e. a -1 for 1983-2002 and -1.63 m w.e. a -1 for 2003-2013. This indicates a clear and regionally consistent acceleration of mass loss over recent decades over the entire European Alps.

Print ISSN: 0094-8276

Electronic ISSN: 1944-8007

Topics: Geosciences , Physics

Published by Wiley-Blackwell on behalf of American Geophysical Union (AGU).

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Therapeutic effect of JAK1/2 blockade on the manifestations of hemophagocytic lymphohistiocytosis in mice (2016)

Maschalidi, S., Sepulveda, F. E., Garrigue, A., Fischer, A., de Saint Basile, G.

American Society of Hematology (ASH)

In: Blood

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Publication Date: 2016-07-08

Description: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome, characterized by severe hyperinflammation and immunopathological manifestations in several tissues. These features result from organ infiltration by overactivated CD8 T-cells and macrophages, which produce high levels of pro-inflammatory cytokines, such as IFN-, TNF-α, IL-6, and IL-18. Recently, several Janus kinase 1/2 (JAK1/2) inhibitors, such as ruxolitinib, have been developed as immunosuppressive agents. They have proven beneficial effects in the treatment of myeloproliferative disorders and inflammatory conditions. To determine whether pharmacological inhibition of the JAK1/2 not only prevents the onset of HLH immunopathology but also is effective against existing HLH, cytotoxicity-impaired Prf1 –/– and Rab27a –/– mice with full-blown HLH syndrome were treated with a clinically relevant dose of ruxolitinib. In vivo, ruxolitinib treatment suppressed signal transducer and activator of transcription 1 activation and led to recovery from HLH manifestations in both murine models. In the Prf1 –/– mice, these beneficial effects were evidenced by a greater survival rate, and in both murine models, they were evidenced by the correction of blood cytopenia and a rapid decrease in serum IL-6 and TNF-α levels. During ruxolitinib treatment, liver tissue damage receded concomitantly with a decrease in the number of infiltrating inflammatory macrophages and an increase in the number of alternatively activated macrophages. In Rab27a –/– mice, central nervous system involvement was significantly reduced by ruxolitinib therapy. Our findings demonstrate that clinically relevant doses of the JAK1/2 inhibitor ruxolitinib suppresses the harmful consequences of macrophage overactivation characterizing HLH in 2 murine models. The results could be readily translated into the clinic for the treatment of primary, and perhaps even secondary, forms of HLH.

Keywords: Immunobiology

Print ISSN: 0006-4971

Electronic ISSN: 1528-0020

Topics: Biology , Medicine

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