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1. Clinical evolution, genetic landscape and trajectories of clonal hematopoiesis in SAMD9/SAMD9L syndromes

Author

Sahoo, S. S., Pastor, V. B., Goodings, C., Voss, R. K., Kozyra, E. J., Szvetnik, A., Noellke, P., Dworzak, M., Stary, J., Locatelli, Franco, Masetti, R., Schmugge, M., De Moerloose, B., Catala, A., Kallay, K., Turkiewicz, D., Hasle, H., Buechner, J., Jahnukainen, K., Ussowicz, M., Polychronopoulou, S., Smith, O. P., Fabri, O., Barzilai, S., de Haas, V., Baumann, I., Schwarz-Furlan, S., Smith, O., Haas, V. D., Gohring, G., Niemeyer, C., Nebral, K., Simonitsch-Kluppp, I., Paepe, P. D., Van Roy, N., Campr, V., Zemanova, Z., Clasen-Linde, E., Plesner, T., Schlegelberger, B., Rudelius, M., Manola, K., Stefanaki, K., Csomor, J., Andrikovics, H., Betts, D., O'Sullivan, M., Zohar, Y., Jeison, M., Vito, R. D., Pasquali, F., Maldyk, J., Haus, O., Alaiz, H., Kjollerstrom, P., Lemos, L. M., Bodova, I., Cermak, M., Plank, L., Gazic, B., Kavcic, M., Podgornik, H., Ros, M. L., Cervera, J., Gengler, C., Tchinda, J., Beverloo, B., Leguit, R., Niewisch, M. R., Sauer, M. G., Burkhardt, B., Lang, P., Bader, P., Beier, R., Muller, I., Albert, M. H., Meisel, R., Schulz, A., Cario, G., Panda, P. K., Wehrle, J., Hirabayashi, S., Derecka, M., Durruthy-Durruthy, R., Yoshimi-Noellke, A., Ku, M., Lebrecht, D., Erlacher, M., Flotho, C., Strahm, B., Niemeyer, C. M., Wlodarski, M. W., Locatelli F. (ORCID:0000-0002-7976-3654), Sahoo, S. S., Pastor, V. B., Goodings, C., Voss, R. K., Kozyra, E. J., Szvetnik, A., Noellke, P., Dworzak, M., Stary, J., Locatelli, Franco, Masetti, R., Schmugge, M., De Moerloose, B., Catala, A., Kallay, K., Turkiewicz, D., Hasle, H., Buechner, J., Jahnukainen, K., Ussowicz, M., Polychronopoulou, S., Smith, O. P., Fabri, O., Barzilai, S., de Haas, V., Baumann, I., Schwarz-Furlan, S., Smith, O., Haas, V. D., Gohring, G., Niemeyer, C., Nebral, K., Simonitsch-Kluppp, I., Paepe, P. D., Van Roy, N., Campr, V., Zemanova, Z., Clasen-Linde, E., Plesner, T., Schlegelberger, B., Rudelius, M., Manola, K., Stefanaki, K., Csomor, J., Andrikovics, H., Betts, D., O'Sullivan, M., Zohar, Y., Jeison, M., Vito, R. D., Pasquali, F., Maldyk, J., Haus, O., Alaiz, H., Kjollerstrom, P., Lemos, L. M., Bodova, I., Cermak, M., Plank, L., Gazic, B., Kavcic, M., Podgornik, H., Ros, M. L., Cervera, J., Gengler, C., Tchinda, J., Beverloo, B., Leguit, R., Niewisch, M. R., Sauer, M. G., Burkhardt, B., Lang, P., Bader, P., Beier, R., Muller, I., Albert, M. H., Meisel, R., Schulz, A., Cario, G., Panda, P. K., Wehrle, J., Hirabayashi, S., Derecka, M., Durruthy-Durruthy, R., Yoshimi-Noellke, A., Ku, M., Lebrecht, D., Erlacher, M., Flotho, C., Strahm, B., Niemeyer, C. M., Wlodarski, M. W., and Locatelli F. (ORCID:0000-0002-7976-3654)

Abstract

Germline SAMD9 and SAMD9L mutations (SAMD9/9Lmut) predispose to myelodysplastic syndromes (MDS) with propensity for somatic rescue. In this study, we investigated a clinically annotated pediatric MDS cohort (n = 669) to define the prevalence, genetic landscape, phenotype, therapy outcome and clonal architecture of SAMD9/9L syndromes. In consecutively diagnosed MDS, germline SAMD9/9Lmut accounted for 8% and were mutually exclusive with GATA2 mutations present in 7% of the cohort. Among SAMD9/9Lmut cases, refractory cytopenia was the most prevalent MDS subtype (90%); acquired monosomy 7 was present in 38%; constitutional abnormalities were noted in 57%; and immune dysfunction was present in 28%. The clinical outcome was independent of germline mutations. In total, 67 patients had 58 distinct germline SAMD9/9Lmut clustering to protein middle regions. Despite inconclusive in silico prediction, 94% of SAMD9/9Lmut suppressed HEK293 cell growth, and mutations expressed in CD34+ cells induced overt cell death. Furthermore, we found that 61% of SAMD9/9Lmut patients underwent somatic genetic rescue (SGR) resulting in clonal hematopoiesis, of which 95% was maladaptive (monosomy 7 ± cancer mutations), and 51% had adaptive nature (revertant UPD7q, somatic SAMD9/9Lmut). Finally, bone marrow single-cell DNA sequencing revealed multiple competing SGR events in individual patients. Our findings demonstrate that SGR is common in SAMD9/9Lmut MDS and exemplify the exceptional plasticity of hematopoiesis in children.

Published
2021

2. Publisher Correction: Clinical evolution, genetic landscape and trajectories of clonal hematopoiesis in SAMD9/SAMD9L syndromes (Nature Medicine, (2021), 27, 10, (1806-1817), 10.1038/s41591-021-01511-6)

Author

Sahoo, S. S., Pastor, V. B., Goodings, C., Voss, R. K., Kozyra, E. J., Szvetnik, A., Noellke, P., Dworzak, M., Stary, J., Locatelli, Franco, Masetti, R., Schmugge, M., De Moerloose, B., Catala, A., Kallay, K., Turkiewicz, D., Hasle, H., Buechner, J., Jahnukainen, K., Ussowicz, M., Polychronopoulou, S., Smith, O. P., Fabri, O., Barzilai, S., de Haas, V., Baumann, I., Schwarz-Furlan, S., Smith, O., Haas, V. D., Gohring, G., Niemeyer, C., Nebral, K., Simonitsch-Kluppp, I., Paepe, P. D., Van Roy, N., Campr, V., Zemanova, Z., Clasen-Linde, E., Plesner, T., Schlegelberger, B., Rudelius, M., Manola, K., Stefanaki, K., Csomor, J., Andrikovics, H., Betts, D., O'Sullivan, M., Zohar, Y., Jeison, M., Vito, R. D., Pasquali, F., Maldyk, J., Haus, O., Alaiz, H., Kjollerstrom, P., Lemos, L. M., Bodova, I., Cermak, M., Plank, L., Gazic, B., Kavcic, M., Podgornik, H., Ros, M. L., Cervera, J., Gengler, C., Tchinda, J., Beverloo, B., Leguit, R., Niewisch, M. R., Sauer, M. G., Burkhardt, B., Lang, P., Bader, P., Beier, R., Muller, I., Albert, M. H., Meisel, R., Schulz, A., Cario, G., Panda, P. K., Wehrle, J., Hirabayashi, S., Derecka, M., Durruthy-Durruthy, R., Yoshimi-Noellke, A., Ku, M., Lebrecht, D., Erlacher, M., Flotho, C., Strahm, B., Niemeyer, C. M., Wlodarski, M. W., Locatelli F. (ORCID:0000-0002-7976-3654), Sahoo, S. S., Pastor, V. B., Goodings, C., Voss, R. K., Kozyra, E. J., Szvetnik, A., Noellke, P., Dworzak, M., Stary, J., Locatelli, Franco, Masetti, R., Schmugge, M., De Moerloose, B., Catala, A., Kallay, K., Turkiewicz, D., Hasle, H., Buechner, J., Jahnukainen, K., Ussowicz, M., Polychronopoulou, S., Smith, O. P., Fabri, O., Barzilai, S., de Haas, V., Baumann, I., Schwarz-Furlan, S., Smith, O., Haas, V. D., Gohring, G., Niemeyer, C., Nebral, K., Simonitsch-Kluppp, I., Paepe, P. D., Van Roy, N., Campr, V., Zemanova, Z., Clasen-Linde, E., Plesner, T., Schlegelberger, B., Rudelius, M., Manola, K., Stefanaki, K., Csomor, J., Andrikovics, H., Betts, D., O'Sullivan, M., Zohar, Y., Jeison, M., Vito, R. D., Pasquali, F., Maldyk, J., Haus, O., Alaiz, H., Kjollerstrom, P., Lemos, L. M., Bodova, I., Cermak, M., Plank, L., Gazic, B., Kavcic, M., Podgornik, H., Ros, M. L., Cervera, J., Gengler, C., Tchinda, J., Beverloo, B., Leguit, R., Niewisch, M. R., Sauer, M. G., Burkhardt, B., Lang, P., Bader, P., Beier, R., Muller, I., Albert, M. H., Meisel, R., Schulz, A., Cario, G., Panda, P. K., Wehrle, J., Hirabayashi, S., Derecka, M., Durruthy-Durruthy, R., Yoshimi-Noellke, A., Ku, M., Lebrecht, D., Erlacher, M., Flotho, C., Strahm, B., Niemeyer, C. M., Wlodarski, M. W., and Locatelli F. (ORCID:0000-0002-7976-3654)

Abstract

In the version of this Article initially published, there were errors in Fig. 1. Specifically, in the top right box in Fig. 1a, the text now reading “n = 25” mistakenly read “125” in the original publication. Further, in the four graphs of Fig. 1e, the colored labels “Wild-type,” “SAMD9/9Lmut” and “GATA2mut” were misaligned to the n and P values at their right. These errors have been corrected in the online version of the article.

Published
2021

3. Case Report: Convalescent Plasma, a Targeted Therapy for Patients with CVID and Severe COVID-19

Author

Van Damme, K.F.A. (Karel F. A.), Tavernier, S.J. (Simon), Van Roy, N. (Nele), De Leeuw, E. (Elisabeth), Declercq, J. (Jozefien), Bosteels, C. (Cédric), Maes, B. (Bastiaan), De Bruyne, M. (Marieke), Bogaert, D.J.A. (Delfien), Bosteels, V. (Victor), Hoste, L. (Levi), Naesens, L. (Leslie), Maes, P. (Piet), Grifoni, A. (Alba), Weiskopf, D. (Daniela), Sette, A. (Alessandro), Depuydt, P. (Pieter), Van Braeckel, E. (Eva), Haerynck, F. (Filomeen), Lambrecht, B.N.M. (Bart), Van Damme, K.F.A. (Karel F. A.), Tavernier, S.J. (Simon), Van Roy, N. (Nele), De Leeuw, E. (Elisabeth), Declercq, J. (Jozefien), Bosteels, C. (Cédric), Maes, B. (Bastiaan), De Bruyne, M. (Marieke), Bogaert, D.J.A. (Delfien), Bosteels, V. (Victor), Hoste, L. (Levi), Naesens, L. (Leslie), Maes, P. (Piet), Grifoni, A. (Alba), Weiskopf, D. (Daniela), Sette, A. (Alessandro), Depuydt, P. (Pieter), Van Braeckel, E. (Eva), Haerynck, F. (Filomeen), and Lambrecht, B.N.M. (Bart)

Abstract

The disease course of COVID-19 in patients with immunodeficiencies is unclear, as well as the optimal therapeutic strategy. We report a case of a 37-year old male with common variable immunodeficiency disorder and a severe SARS-CoV-2 infection. After administration of convalescent plasma, the patient’s condition improved rapidly. Despite clinical recovery, viral RNA remained detectable up to 60 days after onset of symptoms. We propose that convalescent plasma might be considered as a treatment option in patients with CVID and severe COVID-19. In addition, in patients with immunodeficiencies, a different clinical course is possible, with prolonged viral shedding.

Published
2020
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4. Case Report: Convalescent Plasma, a Targeted Therapy for Patients with CVID and Severe COVID-19

Author

van Damme, KFA, Tavernier, S, van Roy, N, de Leeuw, E, Declercq, J, Bosteels, C, Maes, B, de Bruyne, M, Bogaert, D, Bosteels, V, Hoste, L, Naesens, L, Maes, P, Grifoni, A, Weiskopf, D, Sette, A, Depuydt, P, Van Braeckel, E, Haerynck, F, Lambrecht, Bart, van Damme, KFA, Tavernier, S, van Roy, N, de Leeuw, E, Declercq, J, Bosteels, C, Maes, B, de Bruyne, M, Bogaert, D, Bosteels, V, Hoste, L, Naesens, L, Maes, P, Grifoni, A, Weiskopf, D, Sette, A, Depuydt, P, Van Braeckel, E, Haerynck, F, and Lambrecht, Bart

Published
2020

5. Differential impact of drugs on the outcome of ETV6-RUNX1 positive childhood B-cell precursor acute lymphoblastic leukaemia: results of the EORTC CLG 58881 and 58951 trials.

Author

Piette, C, Suciu, S, Clappier, E, Bertrand, Y, Drunat, Séverine, Girard, S, Yakouben, Karima, Plat, G, Dastugue, Nicole, Mazingue, F, Grardel, Nathalie, van Roy, N, Uyttebroeck, Anne, Costa, V, Minckes, Odile, Sirvent, Nicolas, Simon, P, Lutz, P, Ferster, Alina, Pluchart, C, Poirée, Marilyne, Freycon, C, Dresse, M-F, Millot, Frédéric, Chantrain, C, van der Werff Ten Bosch, Jutte, Norga, K, Gilotay, C., Rohrlich, P-S, Benoît, Yves, Cavé, H, Piette, C, Suciu, S, Clappier, E, Bertrand, Y, Drunat, Séverine, Girard, S, Yakouben, Karima, Plat, G, Dastugue, Nicole, Mazingue, F, Grardel, Nathalie, van Roy, N, Uyttebroeck, Anne, Costa, V, Minckes, Odile, Sirvent, Nicolas, Simon, P, Lutz, P, Ferster, Alina, Pluchart, C, Poirée, Marilyne, Freycon, C, Dresse, M-F, Millot, Frédéric, Chantrain, C, van der Werff Ten Bosch, Jutte, Norga, K, Gilotay, C., Rohrlich, P-S, Benoît, Yves, and Cavé, H

Abstract

info:eu-repo/semantics/published

Published
2018

6. ZEB2 drives immature T-cell lymphoblastic leukaemia development via enhanced tumour-initiating potential and IL-7 receptor signalling

Author

Goossens, S, Radaelli, E, Blanchet, O, Durinck, K, Van Der Meulen, J, Peirs, S, Taghon, T, Tremblay, CS, Costa, M, Ghahremani, MF, De Medts, J, Bartunkova, S, Haigh, K, Schwab, C, Farla, N, Pieters, T, Matthijssens, F, Van Roy, N, Best, JA, Deswarte, K, Bogaert, P, Carmichael, C, Rickard, A, Suryani, S, Bracken, LS, Alserihi, R, Canté-Barrett, K, Haenebalcke, L, Clappier, E, Rondou, P, Slowicka, K, Huylebroeck, D, Goldrath, AW, Janzen, V, McCormack, MP, Lock, RB, Curtis, DJ, Harrison, C, Berx, G, Speleman, F, Meijerink, JPP, Soulier, J, Van Vlierberghe, P, Haigh, JJ, Goossens, S, Radaelli, E, Blanchet, O, Durinck, K, Van Der Meulen, J, Peirs, S, Taghon, T, Tremblay, CS, Costa, M, Ghahremani, MF, De Medts, J, Bartunkova, S, Haigh, K, Schwab, C, Farla, N, Pieters, T, Matthijssens, F, Van Roy, N, Best, JA, Deswarte, K, Bogaert, P, Carmichael, C, Rickard, A, Suryani, S, Bracken, LS, Alserihi, R, Canté-Barrett, K, Haenebalcke, L, Clappier, E, Rondou, P, Slowicka, K, Huylebroeck, D, Goldrath, AW, Janzen, V, McCormack, MP, Lock, RB, Curtis, DJ, Harrison, C, Berx, G, Speleman, F, Meijerink, JPP, Soulier, J, Van Vlierberghe, P, and Haigh, JJ

Abstract

Early T-cell precursor leukaemia (ETP-ALL) is a high-risk subtype of human leukaemia that is poorly understood at the molecular level. Here we report translocations targeting the zinc finger E-box-binding transcription factor ZEB2 as a recurrent genetic lesion in immature/ETP-ALL. Using a conditional gain-of-function mouse model, we demonstrate that sustained Zeb2 expression initiates T-cell leukaemia. Moreover, Zeb2-driven mouse leukaemia exhibit some features of the human immature/ETP-ALL gene expression signature, as well as an enhanced leukaemia-initiation potential and activated Janus kinase (JAK)/signal transducers and activators of transcription (STAT) signalling through transcriptional activation of IL7R. This study reveals ZEB2 as an oncogene in the biology of immature/ETP-ALL and paves the way towards pre-clinical studies of novel compounds for the treatment of this aggressive subtype of human T-ALL using our Zeb2-driven mouse model.

Published
2015

7. The stratified significance of a historic facade as a basis for a more durable conservation approach

Author

Van Roy, N. (author), Van Balen, K. (author), Verstrynge, E. (author), Naldini, S. (author), Van Roy, N. (author), Van Balen, K. (author), Verstrynge, E. (author), and Naldini, S. (author)

Abstract

In heritage conservation, a gap is often observed between the theory of conservation as a durable process that aims at the preservation of a historic building and the practice of restoration as a single intervention that aims at a fast and convincing result. This paper describes the proposed approach for the conservation of the main façade of the Shoemakers Chapel (in Dutch: Schoenmakerskapel) in Antwerp (Belgium), a listed monument since 1976. It serves as an example of how to develop a durable and realistic approach for the conservation of a sixteenth century façade. The basis for the conservation approach is the understanding that each intervention should take the stratified significance of the historic façade into account. In this paper, it will be shown how to combine a study of the façade from a technical point of view with an analysis of the façade as a carrier of cultural significance.

Published
2015
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8. ZEB2 drives immature T-cell lymphoblastic leukaemia development via enhanced tumour-initiating potential and IL-7 receptor signalling

Author

Goossens, S, Radaelli, E, Blanchet, O, Durinck, K, Van der Meulen, J, Peirs, S, Taghon, T, Tremblay, CS, Costa, M, Ghahremani, MF, De Medts, J, Bartunkova, S, Haigh, K, Schwab, C, Farla, N, Pieters, T, Matthijssens, F, Van Roy, N, Best, JA, Deswarte, K, Bogaert, P, Carmichael, C, Rickard, A, Suryani, S, Bracken, LS, Alserihi, R, Cante-Barret, K, Haenebalcke, L, Clappier, E, Rondou, P, Slowicka, K, Huylebroeck, D, Goldrath, AW, Janzen, V, McCormack, MP, Lock, RB, Curtis, DJ, Harrison, C, Berx, G, Speleman, F, Meijerink, JPP, Soulier, J, Van Vlierberghe, P, Haigh, JJ, Goossens, S, Radaelli, E, Blanchet, O, Durinck, K, Van der Meulen, J, Peirs, S, Taghon, T, Tremblay, CS, Costa, M, Ghahremani, MF, De Medts, J, Bartunkova, S, Haigh, K, Schwab, C, Farla, N, Pieters, T, Matthijssens, F, Van Roy, N, Best, JA, Deswarte, K, Bogaert, P, Carmichael, C, Rickard, A, Suryani, S, Bracken, LS, Alserihi, R, Cante-Barret, K, Haenebalcke, L, Clappier, E, Rondou, P, Slowicka, K, Huylebroeck, D, Goldrath, AW, Janzen, V, McCormack, MP, Lock, RB, Curtis, DJ, Harrison, C, Berx, G, Speleman, F, Meijerink, JPP, Soulier, J, Van Vlierberghe, P, and Haigh, JJ

Abstract

Early T-cell precursor leukaemia (ETP-ALL) is a high-risk subtype of human leukaemia that is poorly understood at the molecular level. Here we report translocations targeting the zinc finger E-box-binding transcription factor ZEB2 as a recurrent genetic lesion in immature/ETP-ALL. Using a conditional gain-of-function mouse model, we demonstrate that sustained Zeb2 expression initiates T-cell leukaemia. Moreover, Zeb2-driven mouse leukaemia exhibit some features of the human immature/ETP-ALL gene expression signature, as well as an enhanced leukaemia-initiation potential and activated Janus kinase (JAK)/signal transducers and activators of transcription (STAT) signalling through transcriptional activation of IL7R. This study reveals ZEB2 as an oncogene in the biology of immature/ETP-ALL and paves the way towards pre-clinical studies of novel compounds for the treatment of this aggressive subtype of human T-ALL using our Zeb2-driven mouse model.

Published
2015

9. Heterogeneous cytogenetic subgroups and outcomes in childhood acute megakaryoblastic leukemia: A retrospective international study

Author

Inaba, H., Zhou, Y., Abla, O., Adachi, S., Auvrignon, A., Beverloo, H. B., De Bont, E., Chang, T. T., Creutzig, U., Dworzak, M., Elitzur, S., Fynn, A., Forestier, E., Hasle, H., Liang, D. C., Lee, V., Locatelli, Franco, Masetti, R., De Moerloose, B., Reinhardt, D., Rodriguez, L., Van Roy, N., Shen, S., Taga, T., Tomizawa, D., Yeoh, A. E. J., Zimmermann, M., Raimondi, S. C., Locatelli F. (ORCID:0000-0002-7976-3654), Inaba, H., Zhou, Y., Abla, O., Adachi, S., Auvrignon, A., Beverloo, H. B., De Bont, E., Chang, T. T., Creutzig, U., Dworzak, M., Elitzur, S., Fynn, A., Forestier, E., Hasle, H., Liang, D. C., Lee, V., Locatelli, Franco, Masetti, R., De Moerloose, B., Reinhardt, D., Rodriguez, L., Van Roy, N., Shen, S., Taga, T., Tomizawa, D., Yeoh, A. E. J., Zimmermann, M., Raimondi, S. C., and Locatelli F. (ORCID:0000-0002-7976-3654)

Abstract

Comprehensive clinical studies of patients with acute megakaryoblastic leukemia (AMKL) are lacking. We performed an international retrospective study on 490 patients (age ≤18 years) with non-Down syndrome de novo AMKL diagnosed from 1989 to 2009. Patients with AMKL (median age 1.53 years) comprised 7.8% of pediatric AML. Five-year event-free (EFS) and overall survival (OS) were 43.7% ± 2.7% and 49.0% ± 2.7%, respectively. Patients diagnosed in 2000 to 2009 were treated with higher cytarabine doses and had better EFS (P = .037) and OS (P = .003) than those diagnosed in 1989 to 1999. Transplantation in first remission did not improve survival. Cytogenetic data were available for 372 (75.9%) patients: hypodiploid (n = 18, 4.8%), normal karyotype (n = 49, 13.2%), pseudodiploid (n = 119, 32.0%), 47 to 50 chromosomes (n = 142, 38.2%), and >50 chromosomes (n = 44, 11.8%). Chromosome gain occurred in 195 of 372 (52.4%) patients: +21 (n = 106, 28.5%), +19 (n = 93, 25.0%), +8 (n = 77, 20.7%). Losses occurred in 65 patients (17.5%): -7 (n = 13, 3.5%). Common structural chromosomal aberrations were t(1;22)(p13;q13) (n = 51, 13.7%) and 11q23 rearrangements (n = 38, 10.2%); t(9;11)(p22;q23) occurred in 21 patients. On the basis of frequency and prognosis, AMKL can be classified to 3 risk groups: good risk-7p abnormalities; poor risk-normal karyotypes, -7, 9p abnormalities including t(9;11)(p22;q23)/MLL-MLLT3, -13/13q-, and -15; and intermediate risk-others including t(1;22)(p13;q13)/OTT-MAL (RBM15-MKL1) and 11q23/MLL except t(9;11). Risk-based innovative therapy is needed to improve patient outcomes.

Published
2015

10. The stratified significance of a historic facade as a basis for a more durable conservation approach

Author

Van Roy, N. (author), Van Balen, K. (author), Verstrynge, E. (author), Naldini, S. (author), Van Roy, N. (author), Van Balen, K. (author), Verstrynge, E. (author), and Naldini, S. (author)

Abstract

In heritage conservation, a gap is often observed between the theory of conservation as a durable process that aims at the preservation of a historic building and the practice of restoration as a single intervention that aims at a fast and convincing result. This paper describes the proposed approach for the conservation of the main façade of the Shoemakers Chapel (in Dutch: Schoenmakerskapel) in Antwerp (Belgium), a listed monument since 1976. It serves as an example of how to develop a durable and realistic approach for the conservation of a sixteenth century façade. The basis for the conservation approach is the understanding that each intervention should take the stratified significance of the historic façade into account. In this paper, it will be shown how to combine a study of the façade from a technical point of view with an analysis of the façade as a carrier of cultural significance.

Published
2015
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11. ZEB2 drives immature T-cell lymphoblastic leukaemia development via enhanced tumour-initiating potential and IL-7 receptor signalling

Author

Goossens, S, Radaelli, E, Blanchet, O, Durinck, K, van der Meulen, J, Peirs, S, Taghon, T, Tremblay, CS, Costa, M (Marco), Ghahremani, MF, De Medts, J, Bartunkova, S, Haigh, K, Schwab, C, Farla, N, Pieters, T, Matthijssens, F, van Roy, N, Best, JA, Deswarte, K, Bogaert, P, Carmichael, C, Rickard, A, Suryani, S, Bracken, LS, Alserihi, R, Canté, Kirsten, Haenebalcke, L, Clappier, E, Rondou, P, Slowicka, K, Huylebroeck, Danny, Goldrath, AW, Janzen, V, McCormack, MP, Lock, RB, Curtis, DJ, Harrison, C, Berx, G, Speleman, F, Meijerink, Jules, Soulier, J, van Vlierberghe, P, Haigh, JJ, Goossens, S, Radaelli, E, Blanchet, O, Durinck, K, van der Meulen, J, Peirs, S, Taghon, T, Tremblay, CS, Costa, M (Marco), Ghahremani, MF, De Medts, J, Bartunkova, S, Haigh, K, Schwab, C, Farla, N, Pieters, T, Matthijssens, F, van Roy, N, Best, JA, Deswarte, K, Bogaert, P, Carmichael, C, Rickard, A, Suryani, S, Bracken, LS, Alserihi, R, Canté, Kirsten, Haenebalcke, L, Clappier, E, Rondou, P, Slowicka, K, Huylebroeck, Danny, Goldrath, AW, Janzen, V, McCormack, MP, Lock, RB, Curtis, DJ, Harrison, C, Berx, G, Speleman, F, Meijerink, Jules, Soulier, J, van Vlierberghe, P, and Haigh, JJ

Abstract

Early T-cell precursor leukaemia (ETP-ALL) is a high-risk subtype of human leukaemia that is poorly understood at the molecular level. Here we report translocations targeting the zinc finger E-box-binding transcription factor ZEB2 as a recurrent genetic lesion in immature/ETP-ALL. Using a conditional gain-of-function mouse model, we demonstrate that sustained Zeb2 expression initiates T-cell leukaemia. Moreover, Zeb2-driven mouse leukaemia exhibit some features of the human immature/ETP-ALL gene expression signature, as well as an enhanced leukaemia-initiation potential and activated Janus kinase (JAK)/signal transducers and activators of transcription (STAT) signalling through transcriptional activation of IL7R. This study reveals ZEB2 as an oncogene in the biology of immature/ETP-ALL and paves the way towards pre-clinical studies of novel compounds for the treatment of this aggressive subtype of human T-ALL using our Zeb2-driven mouse model.

Published
2015

12. Segmental chromosomal alterations lead to a higher risk of relapse in infants with MYCN-non-amplified localised unresectable/disseminated neuroblastoma (a SIOPEN collaborative study).

Author

UCL - (SLuc) Service d'hématologie et d'oncologie pédiatrique, Schleiermacher, G, Michon, J, Ribeiro, A, Pierron, G, Mosseri, V, Rubie, H, Munzer, C, Bénard, J, Auger, N, Combaret, V, Janoueix-Lerosey, I, Pearson, A, Tweddle, D A, Bown, N, Gerrard, M, Wheeler, K, Noguera, R, Villamon, E, Cañete, A, Castel, V, Marques, B, de Lacerda, A, Tonini, G P, Mazzocco, K, Defferrari, R, de Bernardi, B, di Cataldo, A, van Roy, N, Brichard, Bénédicte, Ladenstein, R, Ambros, I, Ambros, P, Beiske, K, Delattre, O, Couturier, J, UCL - (SLuc) Service d'hématologie et d'oncologie pédiatrique, Schleiermacher, G, Michon, J, Ribeiro, A, Pierron, G, Mosseri, V, Rubie, H, Munzer, C, Bénard, J, Auger, N, Combaret, V, Janoueix-Lerosey, I, Pearson, A, Tweddle, D A, Bown, N, Gerrard, M, Wheeler, K, Noguera, R, Villamon, E, Cañete, A, Castel, V, Marques, B, de Lacerda, A, Tonini, G P, Mazzocco, K, Defferrari, R, de Bernardi, B, di Cataldo, A, van Roy, N, Brichard, Bénédicte, Ladenstein, R, Ambros, I, Ambros, P, Beiske, K, Delattre, O, and Couturier, J

Abstract

In infants with stage 4s MYCN-non-amplified NB, a SCA genomic profile identifies patients who will require upfront treatment even in the absence of other clinical indication for therapy, whereas in infants with localised unresectable NB, a genomic profile characterised by the absence of SCA identifies patients in whom treatment reduction might be possible. These findings will be implemented in a future international trial.

Published
2011

13. Modulation of Mir-449a Expression Decreases Cell Viability, Increases Apoptosis and Induces Differentiation in Evi1 Deregulated Leukemia Cells

Author

UCL - Cliniques universitaires Saint-Luc, UCL - MD/BICL - Département de biochimie et de biologie cellulaire, De Weer, A., Poirel, Hélène, Poppe, B., Mestdagh, P., De Preter, K., Van Vlierberghe, P., Van Roy, N., Jeison, M., Cauwelier, B., Verhasselt, B., Philippé, Jan, Noens, L., Vandenberghe, Peter, Lambert, F., De Paepe, A., Dastugue, N., Vandesompele, J., Speleman, F., 14th Annual Meeting of the European-Hematology-Association, UCL - Cliniques universitaires Saint-Luc, UCL - MD/BICL - Département de biochimie et de biologie cellulaire, De Weer, A., Poirel, Hélène, Poppe, B., Mestdagh, P., De Preter, K., Van Vlierberghe, P., Van Roy, N., Jeison, M., Cauwelier, B., Verhasselt, B., Philippé, Jan, Noens, L., Vandenberghe, Peter, Lambert, F., De Paepe, A., Dastugue, N., Vandesompele, J., Speleman, F., and 14th Annual Meeting of the European-Hematology-Association

Published
2009

14. The TCRB-HOXA rearrangement in T-ALL leads to a specific increase of the alternative HOXA10b transcript

Author

UCL, Cauwelier, B., Cave, H., Dastugue, N., Heimann, P., Antoine-Poirel, Helene, Verhasselt, B., Benoit, Y., De Moerloose, B., Van Roy, N., Hagemeijer, Anne, De Paepe, A., Speleman, F., UCL, Cauwelier, B., Cave, H., Dastugue, N., Heimann, P., Antoine-Poirel, Helene, Verhasselt, B., Benoit, Y., De Moerloose, B., Van Roy, N., Hagemeijer, Anne, De Paepe, A., and Speleman, F.

Published
2006

15. Unequivocal delineation of clinicogenetic subgroups and development of a new model for improved outcome prediction in neuroblastoma

Author

Vandesompele, J, Baudis, Michael; https://orcid.org/0000-0002-9903-4248, De Preter, K, Van Roy, N, Ambros, P, Bown, N, Brinkschmidt, C, Christiansen, H, Combaret, V, Lastowska, M, Nicholson, J, O'Meara, A, Plantaz, D, Stallings, R, Brichard, B, Van den Broecke, C, De Bie, S, De Paepe, A, Laureys, G, Speleman, F, Vandesompele, J, Baudis, Michael; https://orcid.org/0000-0002-9903-4248, De Preter, K, Van Roy, N, Ambros, P, Bown, N, Brinkschmidt, C, Christiansen, H, Combaret, V, Lastowska, M, Nicholson, J, O'Meara, A, Plantaz, D, Stallings, R, Brichard, B, Van den Broecke, C, De Bie, S, De Paepe, A, Laureys, G, and Speleman, F

Abstract

PURPOSE: Neuroblastoma is a genetically heterogeneous pediatric tumor with a remarkably variable clinical behavior ranging from widely disseminated disease to spontaneous regression. In this study, we aimed for comprehensive genetic subgroup discovery and assessment of independent prognostic markers based on genome-wide aberrations detected by comparative genomic hybridization (CGH). MATERIALS AND METHODS: Published CGH data from 231 primary untreated neuroblastomas were converted to a digitized format suitable for global data mining, subgroup discovery, and multivariate survival analyses. RESULTS: In contrast to previous reports, which included only a few genetic parameters, we present here for the first time a strategy that allows unbiased evaluation of all genetic imbalances detected by CGH. The presented approach firmly established the existence of three different clinicogenetic subgroups and indicated that chromosome 17 status and tumor stage were the only independent significant predictors for patient outcome. Important new findings were: (1) a normal chromosome 17 status as a delineator of a subgroup of presumed favorable-stage tumors with highly increased risk; (2) the recognition of a survivor signature conferring 100% 5-year survival for stage 1, 2, and 4S tumors presenting with whole chromosome 17 gain; and (3) the identification of 3p deletion as a hallmark of older age at diagnosis. CONCLUSION: We propose a new regression model for improved patient outcome prediction, incorporating tumor stage, chromosome 17, and amplification/deletion status. These findings may prove highly valuable with respect to more reliable risk assessment, evaluation of clinical results, and optimization of current treatment protocols.

Published
2005

16. Proximal deletion of chromosome 21 confirmed by in situ hybridization and molecular studies.

Author

Courtens, Winnie, Petersen, Merete, Noël, Jean Christophe, Flament Durand, Jacqueline, Van Regemorter, Nicole, Delneste, Danielle, Cochaux, Pascale, Verschraegen-Spae, M, Van Roy, N, Speleman, Frank, Courtens, Winnie, Petersen, Merete, Noël, Jean Christophe, Flament Durand, Jacqueline, Van Regemorter, Nicole, Delneste, Danielle, Cochaux, Pascale, Verschraegen-Spae, M, Van Roy, N, and Speleman, Frank

Abstract

Foetal blood sampling was performed at 35 weeks of gestation due to abnormal foetal ultrasound findings. There was apparent monosomy 21 (45,XX,-21) in all mitoses analyzed. The infant died at 37 weeks during delivery. Examination disclosed facial anomalies, clubfeet, hypoplasia of the left urogenital tract, agenesis of corpus callosum, ventricular dilatation, and heterotopias. Reevaluation of the karyotype showed an unbalanced translocation t(1;21) (q44;q22.11) which resulted from a maternal balanced translocation. These findings were confirmed by fluorescence in situ hybridization and molecular studies with chromosome 21 specific markers. The latter showed a proximal deletion of the maternally derived chromosome 21 including all loci from centromere down to the D21S210 locus. This case illustrates the need for complementary cytogenetic and molecular investigations in cases of apparent monosomy 21., Case Reports, Journal Article, Research Support, Non-U.S. Gov't, Review, info:eu-repo/semantics/published

Published
1994

17. Proximal deletion of chromosome 21 confirmed by in situ hybridization and molecular studies.

Author

Courtens, Winnie, Petersen, Merete, Noël, Jean Christophe, Flament Durand, Jacqueline, Van Regemorter, Nicole, Delneste, Danielle, Cochaux, Pascale, Verschraegen-Spae, M, Van Roy, N, Speleman, Frank, Courtens, Winnie, Petersen, Merete, Noël, Jean Christophe, Flament Durand, Jacqueline, Van Regemorter, Nicole, Delneste, Danielle, Cochaux, Pascale, Verschraegen-Spae, M, Van Roy, N, and Speleman, Frank

Abstract

Foetal blood sampling was performed at 35 weeks of gestation due to abnormal foetal ultrasound findings. There was apparent monosomy 21 (45,XX,-21) in all mitoses analyzed. The infant died at 37 weeks during delivery. Examination disclosed facial anomalies, clubfeet, hypoplasia of the left urogenital tract, agenesis of corpus callosum, ventricular dilatation, and heterotopias. Reevaluation of the karyotype showed an unbalanced translocation t(1;21) (q44;q22.11) which resulted from a maternal balanced translocation. These findings were confirmed by fluorescence in situ hybridization and molecular studies with chromosome 21 specific markers. The latter showed a proximal deletion of the maternally derived chromosome 21 including all loci from centromere down to the D21S210 locus. This case illustrates the need for complementary cytogenetic and molecular investigations in cases of apparent monosomy 21., Case Reports, Journal Article, Research Support, Non-U.S. Gov't, Review, info:eu-repo/semantics/published

Published
1994

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