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945 results on '"Schönland, Stefan"'

201. Comparison of Different Upfront Transplant Strategies in Multiple Myeloma - a Large Registry Study from Chronic Malignancies Working Party of EBMT

Author

Schönland, Stefan O., primary, Iacobelli, Simona, primary, Koster, Linda, primary, Blaise, Didier, primary, Potter, Michael, primary, Cornelissen, Jan J, primary, Chevallier, Patrice, primary, Mayer, Jiri, primary, Reményi, Péter, primary, Gribben, John G., primary, Meijer, Ellen, primary, Carpenter, Ben, primary, Collin, Matthew P., primary, Gedde-Dahl, Tobias, primary, Potter, Victoria, primary, Malladi, Ram, primary, Stelljes, Matthias, primary, Bloor, Adrian, primary, Tuglular, Tulin, primary, Weissinger, Florian, primary, Beksac, Meral, primary, Hayden, Patrick J, primary, Yakoub-Agha, Ibrahim, primary, and Kröger, Nicolaus, primary

Published
2019
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202. OP201: A Phase 1/2 Study of Melflufen and Dexamethasone in Patients with Immunoglobulin Light Chain (AL) Amyloidosis

Author

Palladini, Giovanni, primary, Schönland, Stefan O., additional, Lentzsch, Suzanne, additional, Cibeira, M. Teresa, additional, Hajek, Roman, additional, Jaccard, Arnaud, additional, Jamroziak, Krzysztof, additional, Kastritis, Eftathios, additional, Sanchorawala, Vaishali, additional, Schjesvold, Fredrik H., additional, Wechalekar, Ashutosh D, additional, Thuresson, Sara, additional, Harmenberg, Johan, additional, Byrne, Catriona, additional, and Merlini, Giampaolo, additional

Published
2019
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203. High-Throughput Immunofluorescence and Electron Tomography to Characterize Centrosomal Aberrations in Plasma Cell Neoplasia

Author

Dittrich, Tobias, primary, Schorb, Martin, additional, Haberbosch, Isabella, additional, Bausch, Elena, additional, Börmel, Mandy, additional, Cosenza, Marco, additional, Goldschmidt, Hartmut, additional, Müller-Tidow, Carsten, additional, Raab, Marc S, additional, Hegenbart, Ute, additional, Schönland, Stefan O., additional, Schwab, Yannick, additional, and Krämer, Alwin, additional

Published
2019
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205. Maintaining the Cellular Anti-Viral and Anti-Leukemic Activities in GvHD Patients Undergoing Extracorporeal Photophoresis Therapy

Author

Gößmann, Ruben A., primary, Wang, Lei, additional, Ni, Ming, additional, Yang, Mingya, additional, Luft, Thomas, additional, Hegenbart, Ute, additional, Schönland, Stefan O., additional, Wuchter, Patrick, additional, Eckstein, Volker, additional, Krüger, William Hermann, additional, Yerushalmi, Ronit, additional, Beider, Katia, additional, Nagler, Arnon, additional, Müller-Tidow, Carsten, additional, Dreger, Peter, additional, Schmitt, Michael, additional, and Schmitt, Anita, additional

Published
2019
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206. Results of the Phase 3 VITAL Study of NEOD001 (Birtamimab) Plus Standard of Care in Patients with Light Chain (AL) Amyloidosis Suggest Survival Benefit for Mayo Stage IV Patients

Author

Gertz, Morie A, primary, Cohen, Adam D., additional, Comenzo, Raymond L, additional, Du Mond, Charles, additional, Kastritis, Eftathios, additional, Landau, Heather J, additional, Libby, Edward N., additional, Liedtke, Michaela, additional, Merlini, Giampaolo, additional, Sanchorawala, Vaishali, additional, Schönland, Stefan O., additional, Wechalekar, Ashutosh D, additional, Zonder, Jeffrey A., additional, and Kinney, Gene, additional

Published
2019
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207. Safety and Efficacy of Autologous Stem Cell Transplantation in Dialysis-Dependent Myeloma Patients - the Diadem Study from the Chronic Malignancies Working Party of the EBMT

Author

Waszczuk-Gajda, Anna, primary, Wang, Junfeng, additional, de Wreede, Liesbeth C., additional, Sirait, Tiarlan, additional, Ozkurt, Zubeyde Nur, additional, Snowden, John A, additional, Dreger, Peter, additional, Illés, Árpád, additional, Arat, Mutlu, additional, Bulabois, Claude-Eric, additional, Baurmann, Herrad, additional, Sever, Matjaz, additional, Basak, Grzegorz W, additional, Nikolousis, Manos, additional, Potter, Victoria, additional, Schroyens, Wilfried, additional, Gyan, Emmanuel, additional, Van Gorkom, Gwendolyn, additional, de la Serna, Javier, additional, Guffroy, Blandine, additional, Hayden, Patrick J, additional, Beksac, Meral, additional, Schönland, Stefan O., additional, and Yakoub-Agha, Ibrahim, additional

Published
2019
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208. Eight novel loci implicate shared genetic etiology in multiple myeloma, AL amyloidosis, and monoclonal gammopathy of unknown significance

Author

Chattopadhyay, Subhayan, primary, Thomsen, Hauke, additional, Weinhold, Niels, additional, Meziane, Iman, additional, Huhn, Stefanie, additional, da Silva Filho, Miguel Inacio, additional, Vodicka, Pavel, additional, Vodickova, Ludmila, additional, Hoffmann, Per, additional, Nöthen, Markus M., additional, Jöckel, Karl-Heinz, additional, Schmidt, Börge, additional, Landi, Stefano, additional, Hajek, Roman, additional, Hallmans, Göran, additional, Pettersson-Kymmer, Ulrika, additional, Ohlsson, Claes, additional, Milani, Paolo, additional, Merlini, Giampaolo, additional, Rowcieno, Dorota, additional, Hawkins, Philip, additional, Hegenbart, Ute, additional, Palladini, Giovanni, additional, Wechalekar, Ashutosh, additional, Schönland, Stefan O., additional, Houlston, Richard, additional, Goldschmidt, Hartmut, additional, Hemminki, Kari, additional, and Försti, Asta, additional

Published
2019
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211. Shaping of CD56bri Natural Killer Cells in Patients With Steroid-Refractory/Resistant Acute Graft-vs.-Host Disease via Extracorporeal Photopheresis

Author

Ni, Ming, Wang, Lei, Yang, Mingya, Neuber, Brigitte, Sellner, Leopold, Hückelhoven-Krauss, Angela, Schubert, Maria-Luisa, Luft, Thomas, Hegenbart, Ute, Schönland, Stefan, Wuchter, Patrick, Chen, Bao-an, Eckstein, Volker, Krüger, William, Yerushalmi, Ronit, Beider, Katia, Nagler, Arnon, Müller-Tidow, Carsten, Dreger, Peter, Schmitt, Michael, and Schmitt, Anita

Subjects
Immunology, Immunology and Allergy
Published
2019
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216. Real-world outcomes in non-endemic hereditary transthyretin amyloidosis with polyneuropathy: a 20-year German single-referral centre experience.

Author

Ungerer, Matthias N., Hund, Ernst, Purrucker, Jan C., Huber, Laura, Kimmich, Christoph, aus dem Siepen, Fabian, Hein, Selina, Kristen, Arnt V., Hinderhofer, Katrin, Kollmer, Jennifer, Schönland, Stefan, Hegenbart, Ute, and Weiler, Markus

Subjects
CARDIAC amyloidosis, AMYLOIDOSIS, TRANSTHYRETIN, POLYNEUROPATHIES, NEUROLOGICAL disorders, BRAIN natriuretic factor
Abstract

Hereditary transthyretin amyloidosis is caused by pathogenic variants in the TTR gene and typically manifests, alongside cardiac and other organ dysfunctions, with a rapidly progressive sensorimotor and autonomic polyneuropathy (ATTRv-PN) leading to severe disability. While most prospective studies have focussed on endemic ATTRv-PN, real-world data on non-endemic, mostly late-onset ATTRv-PN are limited. This retrospective study investigated ATTRv-PN patients treated at the Amyloidosis Centre of Heidelberg University Hospital between November 1999 and July 2020. Clinical symptoms, survival, prognostic factors and efficacy of treatment with tafamidis were analysed. Neurologic outcome was assessed using the Coutinho ATTRv-PN stages, and the Peripheral Neuropathy Disability (PND) score. Of 346 subjects with genetic TTR variants, 168 patients had symptomatic ATTRv-PN with 32 different TTR variants identified. Of these, 81.6% had the late-onset type of ATTRv-PN. Within a mean follow-up period of 4.1 ± 2.8 years, 40.5% of patients died. Baseline plasma N-terminal prohormone of brain natriuretic peptide (NT-proBNP) ≥900 ng/l (HR 3.259 [1.421–7.476]; p =.005) was the main predictor of mortality in multivariable analysis. 64 patients were treated with tafamidis and presented for regular follow-up examinations. The therapeutic benefit of tafamidis was more pronounced when treatment was started early in ATTRv-PN stage 1 (PND scores II vs. I; HR 2.718 [1.258–5.873]; p =.011). In non-endemic, mostly late-onset ATTRv-PN, cardiac involvement assessed by NT-proBNP is a strong prognosticator for overall survival. Long-term treatment with tafamidis is safe and efficacious. Neurologic disease severity at the start of treatment is the main predictor for ATTRv-PN progression on tafamidis. [ABSTRACT FROM AUTHOR]

Published
2021
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217. CD38 as Immunotherapeutic Target in Light Chain Amyloidosis and Multiple Myeloma—Association With Molecular Entities, Risk, Survival, and Mechanisms of Upfront Resistance

Author

Seckinger, Anja, Hillengass, Jens, Emde, Martina, Beck, Susanne, Kimmich, Christoph, Dittrich, Tobias, Hundemer, Michael, Jauch, Anna, Hegenbart, Ute, Raab, Marc-Steffen, Ho, Anthony D, Schönland, Stefan, Hose, Dirk, Hematology, and Basic (bio-) Medical Sciences

Subjects
multiple myeloma, amyloidosis, lcsh:Immunologic diseases. Allergy, alternative splicing, hematology, immune system diseases, hemic and lymphatic diseases, Immunology, immunotherapy, lcsh:RC581-607, survival, CD38, Original Research
Abstract

Monoclonal antibodies against the cell surface antigen CD38, e.g., isatuximab, daratumumab, or Mor202, have entered the therapeutic armamentarium in multiple myeloma due to single agent overall response rates of 29 vs. 36 vs. 31%, effectivity in combination regimen, e.g., with lenalidomide or bortezomib plus dexamethasone, and tolerable side effects. Despite clinical use, many questions remain. In this manuscript, we address three of these: first, upfront CD38 target-expression in AL-amyloidosis, monoclonal gammopathy of unknown significance (MGUS), asymptomatic, symptomatic, and relapsed multiple myeloma. Second, relation of CD38-expression to survival, disease stages, molecular entities, and high-risk definitions. Third, alternative splicing or lack of CD38-expression as potential mechanisms of upfront resistance. We assessed CD138-purified plasma cell samples from 196 AL-amyloidosis, 62 MGUS, 259 asymptomatic, 764 symptomatic, and 90 relapsed myeloma patients, including longitudinal pairs of asymptomatic/symptomatic (n = 34) and symptomatic/relapsed myeloma (n = 57) regarding interphase fluorescence in situ hybridization (n = 1,380), CD38-expression by gene expression profiling (n = 1,371), RNA-sequencing (n = 593), and flow cytometry (n = 800). Samples of normal bone marrow plasma cells (n = 10), memory B-cells (n = 9), polyclonal plasmablastic cells (n = 9), and human myeloma cell lines (n = 54) were used as comparators. CD38 was expressed in all malignant plasma cell samples, but significantly lower compared to normal plasma cells with small but significant downregulation in longitudinal sample pairs. Higher CD38 expression was associated with the presence of t(4;14) and high-risk according to the UAMS70-gene score, lower expression was associated with del17p13 and hyperdiploidy in symptomatic myeloma as well as t(11;14) in asymptomatic myeloma. Higher CD38-expression was associated with slower progression to symptomatic and relapsed myeloma and better overall survival in the latter two entities. CD38 expression, t(4;14), del17p13, and gain of 1q21 are independently prognostic in multivariate analysis. By contrast, high CD38-expression is associated with adverse survival in AL-amyloidosis. Regarding mechanisms of upfront anti-CD38-treatment resistance, lack of CD38-expression and alternative splicing of receptor binding-sites could be excluded. Here, of the two protein coding CD38-transcripts CD38-001 (eight-exon, full length) and CD38-005 (truncated), CD38-001 conveyed >97% of reads spanning the respective CD38 splice junction.

Published
2018

219. Bortezomib-based induction followed by stem cell transplantation in light chain amyloidosis: results of the multicenter HOVON 104 trial

Author

Minnema, Monique C., primary, Nasserinejad, Kazem, additional, Hazenberg, Bouke, additional, Hegenbart, Ute, additional, Vlummens, Philip, additional, Ypma, Paula F., additional, Kröger, Nicolaus, additional, Wu, Ka Lung, additional, Kersten, Marie Jose, additional, Schaafsma, M. Ron, additional, Croockewit, Sandra, additional, de Waal, Esther, additional, Zweegman, Sonja, additional, Tick, Lidwien, additional, Broijl, Annemieke, additional, Koene, Harry, additional, Bos, Gerard, additional, Sonneveld, Pieter, additional, and Schönland, Stefan, additional

Published
2019
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221. Performance analysis of AL amyloidosis cardiac biomarker staging systems with special focus on renal failure and atrial arrhythmia

Author

Dittrich, Tobias, primary, Benner, Axel, additional, Kimmich, Christoph, additional, Siepen, Fabian aus dem, additional, Veelken, Kaya, additional, Kristen, Arnt V., additional, Bochtler, Tilmann, additional, Katus, Hugo A., additional, Müller-Tidow, Carsten, additional, Hegenbart, Ute, additional, and Schönland, Stefan O., additional

Published
2019
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222. Treosulfan Conditioning for Allogeneic Transplantation in Multiple Myeloma Improved Overall Survival in Upfront Hematopoietic Stem Cell Transplantation — a Large Retrospective Study By the Chronic Malignancies Working Party of the EBMT

Author

Gran, Charlotte, primary, Wang, Junfeng, additional, Nahi, Hareth, additional, Koster, Linda, additional, Gahrton, Goesta, additional, Wolschke, Christine, additional, Einsele, Hermann, additional, Itälä-Remes, Maija, additional, Blaise, Didier, additional, Niederwieser, Dietger, additional, Meijer, Ellen, additional, Petersen, Eefke J., additional, de Wreede, Liesbeth C., additional, Ljungman, Per, additional, Bourhis, Jean-Henri, additional, Veelken, Hendrik, additional, Beelen, Dietrich W., additional, Stelljes, Matthias, additional, Gerbitz, Armin, additional, Ceballos, Patrice, additional, Finke, Jürgen, additional, Passweg, Jakob R., additional, Milpied, Noel-Jean, additional, Bornhäuser, Martin, additional, Yakoub-Agha, Ibrahim, additional, Schönland, Stefan, additional, Garderet, Laurent, additional, and Kroeger, Nicolaus, additional

Published
2018
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223. Impact of Upfront Stem Cell Transplantation in Newly Diagnosed Multiple Myeloma with Del(17) and t(4;14): A Report from the EBMT Chronic Malignancies Working Party

Author

Gagelmann, Nico, primary, Eikema, Dirk-Jan, additional, Iacobelli, Simona, additional, Schoemans, Helene, additional, Koster, Linda, additional, Caillot, Denis, additional, Blaise, Didier, additional, Reményi, Péter, additional, Bulabois, Claude-Eric, additional, Passweg, Jakob R., additional, Leleu, Xavier, additional, Zver, Samo, additional, Kobbe, Guido, additional, Rambaldi, Alessandro, additional, Chevallier, Patrice, additional, Ringhoffer, Mark, additional, Martin, Alexander M, additional, Salmenniemi, Urpu, additional, Poire, Xavier, additional, Lenhoff, Stig, additional, Pioltelli, Pietro, additional, Mordini, Nicola, additional, Nahi, Hareth, additional, Schönland, Stefan, additional, Garderet, Laurent, additional, Yakoub-Agha, Ibrahim, additional, and Kroeger, Nicolaus, additional

Published
2018
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224. Pomalidomide and Dexamethasone Grant Rapid Hematologic Responses in Patients with Relapsed and Refractory AL Amyloidois: A European Retrospective Series of 150 Patients

Author

Milani, Paolo, primary, Sharpley, Faye, additional, Schönland, Stefan, additional, Basset, Marco, additional, Mahmood, Shameem, additional, Kimmich, Christoph, additional, Foli, Andrea, additional, Sachchithanantham, Sajitha, additional, Merlini, Giampaolo, additional, Wechalekar, Ashutosh D., additional, Palladini, Giovanni, additional, and Hegenbart, Ute, additional

Published
2018
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225. Incidence of Acute Graft-Versus-Host Disease and Survival after Allogeneic Hematopoietic Cell Transplantation over Time: A Study from the Transplant Complications and Chronic Malignancies Working Party of the EBMT

Author

Greinix, Hildegard T., primary, Eikema, Dirk-Jan, additional, Koster, Linda, additional, Penack, Olaf, additional, Yakoub-Agha, Ibrahim, additional, Montoto, Silvia, additional, Chabannon, Christian, additional, Styczynski, Jan, additional, Nagler, Arnon, additional, Robin, Marie, additional, Robinson, Stephen, additional, Chalandon, Yves, additional, Mikulska, Malgorzata, additional, Schönland, Stefan, additional, Peric, Zinaida, additional, Ruggeri, Annalisa, additional, Lanza, Francesco, additional, Mohty, Mohamad, additional, Basak, Grzegorz W, additional, and Nicolaus, Kroger, additional

Published
2018
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226. No Inhibition of Anti-Viral and Anti-Leukemia Effects By Extracorporeal Photopheresis Therapy

Author

NI, Ming, primary, Wang, Lei, additional, Hückelhoven, Angela, additional, Sellner, Leopold, additional, Neuber, Brigitte, additional, Hoffmann, Jean-Marc, additional, Luft, Thomas, additional, Hegenbart, Ute, additional, Schönland, Stefan, additional, Wuchter, Patrick, additional, Eckstein, Volker, additional, Krüger, William Hermann, additional, Yerushalmi, Ronit, additional, Nagler, Arnon, additional, Hilgendorf, Inken, additional, Müller-Tidow, Carsten, additional, Ho, Anthony D, additional, Dreger, Peter, additional, Schmitt, Michael, additional, and Schmitt, Anita, additional

Published
2018
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227. Results of Autologous and Allogeneic Transplantation in Patients with Primary Plasma Cell Leukemia: A Large Retrospective Analysis of the Chronic Malignancies Working Party of the EBMT

Author

Lawless, Sarah, primary, Iacobelli, Simona, additional, Knelange, Nina, additional, Chevallier, Patrice, additional, Blaise, Didier, additional, Milpied, Noel-Jean, additional, Foà, Robin, additional, Cornelissen, Jan J, additional, Lioure, Bruno, additional, Potter, Victoria, additional, Poire, Xavier, additional, Petersen, Eefke J., additional, Collin, Matthew P., additional, Lenhoff, Stig, additional, Snowden, John, additional, Santarone, Stella, additional, Wilson, Keith, additional, Guimaraes, Jose E., additional, Dreger, Peter, additional, Schipperus, Martin R., additional, Morris, Curly, additional, Schönland, Stefan, additional, Yakoub-Agha, Ibrahim, additional, Garderet, Laurent, additional, and Nicolaus, Kroger, additional

Published
2018
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228. AL Amyloidosis — Pathogenesis and Prognosis Are Determined By the Amyloidogenic Potential of the Light Chain and the Molecular Characteristics of Malignant Plasma Cells

Author

Seckinger, Anja, primary, Hegenbart, Ute, additional, Beck, Susanne, additional, Emde, Martina, additional, Bochtler, Tilmann, additional, Kimmich, Christoph, additional, Müller-Tidow, Carsten, additional, Jauch, Anna, additional, Schönland, Stefan, additional, and Hose, Dirk, additional

Published
2018
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229. Cytogenetic intraclonal heterogeneity of plasma cell dyscrasia in AL amyloidosis as compared with multiple myeloma

Author

Bochtler, Tilmann, primary, Merz, Maximilian, additional, Hielscher, Thomas, additional, Granzow, Martin, additional, Hoffmann, Korbinian, additional, Krämer, Alwin, additional, Raab, Marc-Steffen, additional, Hillengass, Jens, additional, Seckinger, Anja, additional, Kimmich, Christoph, additional, Dittrich, Tobias, additional, Müller-Tidow, Carsten, additional, Hose, Dirk, additional, Goldschmidt, Hartmut, additional, Hegenbart, Ute, additional, Jauch, Anna, additional, and Schönland, Stefan O., additional

Published
2018
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230. Modulation of B Cells and Homing Marker on NK Cells Through Extracorporeal Photopheresis in Patients With Steroid-Refractory/Resistant Graft-Vs.-Host Disease Without Hampering Anti-viral/Anti-leukemic Effects

Author

Wang, Lei, primary, Ni, Ming, additional, Hückelhoven-Krauss, Angela, additional, Sellner, Leopold, additional, Hoffmann, Jean-Marc, additional, Neuber, Brigitte, additional, Luft, Thomas, additional, Hegenbart, Ute, additional, Schönland, Stefan, additional, Kleist, Christian, additional, Sill, Martin, additional, Chen, Bao-an, additional, Wuchter, Patrick, additional, Eckstein, Volker, additional, Krüger, William, additional, Hilgendorf, Inken, additional, Yerushalmi, Ronit, additional, Nagler, Arnon, additional, Müller-Tidow, Carsten, additional, Ho, Anthony D., additional, Dreger, Peter, additional, Schmitt, Michael, additional, and Schmitt, Anita, additional

Published
2018
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231. A Novel Early Relapse Prediction Score Based on Age, ISS and Disease Status at the Time of Transplant in Patients with Newly Diagnosed Multiple Myeloma. a Study of the EBMT Chronic Malignancies Working Party

Author

Beksac, Meral, Iacobelli, Simona, Koster, Linda, Blaise, Didier, Cornelissen, Jan J., Reményi, Péter, Leleu, Xavier, Gedde-Dahl, Tobias, Poire, Xavier, Griskevicius, Laimonas, Forcade, Edouard, Lenhoff, Stig, Rabin, Neil K, Kobbe, Guido, Netelenbos, Tanja, Arcese, William, Mear, Jean-Baptiste, Engelhardt, Monika, Pabst, Thomas, Schaap, Nicolaas, Sossa, Claudia Lucia, Elmaagacli, Ahmet, Hayden, Patrick John, Schoenland, Stefan, and Yakoub-Agha, Ibrahim

Published
2021
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232. Cryo-EM reveals structural breaks in a patient-derived amyloid fibril from systemic AL amyloidosis.

Author

Radamaker, Lynn, Baur, Julian, Huhn, Stefanie, Haupt, Christian, Hegenbart, Ute, Schönland, Stefan, Bansal, Akanksha, Schmidt, Matthias, and Fändrich, Marcus

Subjects
AMYLOIDOSIS, AMYLOID, PROTEIN folding, CARDIAC patients
Abstract

Systemic AL amyloidosis is a debilitating and potentially fatal disease that arises from the misfolding and fibrillation of immunoglobulin light chains (LCs). The disease is patient-specific with essentially each patient possessing a unique LC sequence. In this study, we present two ex vivo fibril structures of a λ3 LC. The fibrils were extracted from the explanted heart of a patient (FOR005) and consist of 115-residue fibril proteins, mainly from the LC variable domain. The fibril structures imply that a 180° rotation around the disulfide bond and a major unfolding step are necessary for fibrils to form. The two fibril structures show highly similar fibril protein folds, differing in only a 12-residue segment. Remarkably, the two structures do not represent separate fibril morphologies, as they can co-exist at different z-axial positions within the same fibril. Our data imply the presence of structural breaks at the interface of the two structural forms. Systemic AL amyloidosis is a protein misfolding disease caused by the aggregation and fibrillation of immunoglobulin light chains (LCs). Here, the authors present the cryo-EM structures of λ3 LC-derived amyloid fibrils that were isolated from patient tissue and they observe structural breaks, where the two different fibril structures co-exist at different z-axial positions within the same fibril. [ABSTRACT FROM AUTHOR]

Published
2021
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234. Venetoclax induces deep hematologic remissions in t(11;14) relapsed/refractory AL amyloidosis.

Author

Premkumar, Vikram J., Lentzsch, Suzanne, Pan, Samuel, Bhutani, Divaya, Richter, Joshua, Jagannath, Sundar, Liedtke, Michaela, Jaccard, Arnaud, Wechalekar, Ashutosh D., Comenzo, Raymond, Sanchorawala, Vaishali, Royer, Bruno, Rosenzweig, Michael, Valent, Jason, Schönland, Stefan, Fonseca, Rafael, Wong, Sandy, and Kapoor, Prashant

Subjects
MULTIPLE myeloma treatment, AMYLOIDOSIS treatment, DRUG efficacy, DISEASE remission, DISEASE relapse, DRUG side effects, STEM cell transplantation
Abstract

Venetoclax is efficacious in relapsed/refractory t(11;14) multiple myeloma, thus warranting investigation in light-chain amyloidosis (AL). This retrospective cohort includes 43 patients with previously treated AL, from 14 centers in the US and Europe. Thirty-one patients harbored t(11;14), 11 did not, and one t(11;14) status was unknown. Patients received a venetoclax-containing regimen for at least one 21- or 28-day cycle; the median prior treatments was three. The hematologic response rate for all patients was 68%; 63% achieved VGPR/CR. t(11;14) patients had higher hematologic response (81% vs. 40%) and higher VGPR/CR rate (78% vs. 30%, odds ratio: 0.12, 95% CI 0.02–0.62) than non-t(11;14) patients. For the unsegregated cohort, median progression-free survival (PFS) was 31.0 months and median OS was not reached (NR). For t(11;14), median PFS was NR and for non-t(11;14) median PFS was 6.7 months (HR: 0.14, 95% CI 0.04–0.53). Multivariate analysis incorporating age, sex, prior lines of therapy, and disease stage suggested a risk reduction for progression or death in t(11;14) patients. Median OS was NR for either subgroup. The organ response rate was 38%; most responders harbored t(11;14). Grade 3 or higher adverse events occurred in 19% with 7% due to infections. These promising results require confirmation in a randomized clinical trial. [ABSTRACT FROM AUTHOR]

Published
2021
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236. P352: PROPHYLACTIC AND PREEMPTIVE USE OF DLI COMPARE FAVOURABLY TO THEIR THERAPEUTIC USE IN ADULT ACUTE LYMPHOBLASTIC LEUKEMIA (ALL) – RESULTS OF A MULTICENTRE RETROSPECTIVE STUDY.

Author

Haupt, Maik, Jäkel, Nadja, Wachsmuth, Maria, Stadler, Michael, Beelen, Dietrich, Finke, Juergen, Alakel, Nael, Hemmati, Philipp, Schönland, Stefan, Bethge, Wolfgang, Wulf, Gerald, Wolff, Daniel, Maria Tischer, Johanna, Wagner‐Drouet, Eva, Brandt, Anna, Kröger, Nicolaus, Vucinic, Vladan, Bärmann, Ben‐Niklas, Brüggemann, Monika, and Gromann, Cora

Published
2023
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237. S198: EFFICACY AND SAFETY OF BELANTAMAB MAFODOTIN MONOTHERAPY IN PATIENTS WITH RELAPSED OR REFRACTORY LIGHT CHAIN AMYLOIDOSIS: A PHASE 2 STUDY BY THE EUROPEAN MYELOMA NETWORK.

Author

Kastritis, Efstathios, Palladini, Giovanni, Dimopoulos, Meletios A., Jaccard, Arnaud, Merlini, Giampaolo, Theodorakakou, Foteini, Fotiou, Despina, Minnema Minnema, Monique C., Wechalekar, Ashutosh, Papachristou, Elena, Manousou, Kyriaki, Sonneveld, Pieter, and Schönland, Stefan

Published
2023
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238. Search for AL amyloidosis risk factors using Mendelian randomization

Author

Saunders, Charlie N., Chattopadhyay, Subhayan, Huhn, Stefanie, Weinhold, Niels, Hoffmann, Per, Nöthen, Markus M., Jöckel, Karl-Heinz, Schmidt, Börge, Landi, Stefano, Goldschmidt, Hartmut, Milani, Paolo, Merlini, Giampaolo, Rowcieno, Dorota, Hawkins, Philip, Hegenbart, Ute, Palladini, Giovanni, Wechalekar, Ashutosh, Schönland, Stefan O., Försti, Asta, Houlston, Richard, and Hemminki, Kari

Abstract

•AL amyloidosis risk factors are poorly understood, but MR can be used as a tool to find novel risk factors.•The present findings suggest that increased blood monocyte counts and TNFRSF proteins play roles in AL amyloidosis etiology.

Published
2021
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239. Centre characteristics and procedure-related factors have an impact on outcomes of allogeneic transplantation for patients with CLL: a retrospective analysis from the European Society for Blood and Marrow Transplantation (EBMT)

Author

Schetelig, Johannes, de Wreede, Liesbeth C., Andersen, Niels S., Moreno, Carol, van Gelder, Michel, Vitek, Antonin, Karas, Michal, Michallet, Mauricette, Machaczka, Maciej, Gramatzki, Martin, Beelen, Dietrich, Finke, Jürgen, Delgado, Julio, Volin, Liisa, Passweg, Jakob, Dreger, Peter, Schaap, Nicolaas, Wagner, Eva, Henseler, Anja, van Biezen, Anja, Bornhäuser, Martin, Iacobelli, Simona, Putter, Hein, Schönland, Stefan O., Kröger, Nicolaus, Esteve, Jordi, Ljungman, Per, de Witte, Theo, Stelljes, Matthias, Sierra, Jorge, Socié, Gerard, Ganser, Arnold, Wulf, Gerhard G., Deconinck, Eric, Faber, Edgar, Feguex, Nathalie, Gedde-Dahl, Tobias, Kolbe, Karin, Chalandon, Yves, Krüger, William, Huynh, Anne, Bourhis, Jean Henri, Schouten, Harry, Ribera Santasusana, Josep M., Interne Geneeskunde, MUMC+: MA Hematologie (9), RS: GROW - R3 - Innovative Cancer Diagnostics & Therapy, and Chalandon, Yves

Subjects
Oncology, Male, Transplantation Conditioning, Cancer development and immune defence Radboud Institute for Molecular Life Sciences [Radboudumc 2], Chronic lymphocytic leukemia, Medizin, QUALITY MANAGEMENT-SYSTEM, Kaplan-Meier Estimate, risk factor analysis, GUIDELINES, Biochemistry, allogeneic stem cell transplantation, centre effects, chronic lymphocytic leukaemia, frailties, Transplantation Conditioning / methods, 0302 clinical medicine, Recurrence, Risk Factors, Leukemia, Lymphocytic, Chronic, B-Cell / mortality, Registries, Delivery of Health Care / statistics & numerical data, IBRUTINIB, ddc:616, 0303 health sciences, ALEMTUZUMAB, Hazard ratio, Hematopoietic Stem Cell Transplantation, Professional Practice, Hematology, Middle Aged, 3. Good health, Europe, 030220 oncology & carcinogenesis, ABT-199, Cohort, SURVIVAL, Alemtuzumab, Female, Europe / epidemiology, medicine.drug, Adult, medicine.medical_specialty, Allogeneic transplantation, Immunology, Lower risk, DIAGNOSIS, Settore MED/01 - Statistica Medica, 03 medical and health sciences, Leukemia, Lymphocytic, Chronic, B-Cell / therapy, Hematopoietic Stem Cell Transplantation / methods, Internal medicine, medicine, Humans, Karnofsky Performance Status, 030304 developmental biology, Aged, Retrospective Studies, business.industry, Proportional hazards model, CHRONIC LYMPHOCYTIC-LEUKEMIA, Retrospective cohort study, STEM-CELL TRANSPLANTATION, Cell Biology, medicine.disease, Leukemia, Lymphocytic, Chronic, B-Cell, Confidence interval, Surgery, Professional Practice / statistics & numerical data, Transplantation, business, Delivery of Health Care, 030215 immunology, RESPONSES
Abstract

Introduction:Even in the era of novel targeted therapies for the treatment of Chronic Lymphocytic Leukemia (CLL) patients, such as BTK, PI3K and BCL2 inhibitors, allogeneic hematopoietic stem cell transplantations (alloHCT) will remain an important treatment option for a subset of patients with very high risk CLL. The current study focused on the impact of center and procedure-related factors on outcomes after alloHCT, taking into account the impact of patient- and disease-related risk factors. Patients and Methods:Data of 684 CLL patients who received a first alloHCT between 2000 and 2011 were analyzed. Their data were collected as part of the EBMT CLL Data Quality Initiative. Outcomes of interest were Event-Free Survival (EFS) up to 5 years after transplantation and mortality in the first 100 days after alloHCT. Outcomes were analyzed by means of the Kaplan-Meier method and Cox proportional hazards models with a frailty (random effects) component to take into account unexplained center heterogeneity. The following factors describing center characteristics or the transplant procedure were analyzed: experience in alloHCT in general and, for CLL specifically, accreditation by the Joint Accreditation Committee-ISCT & EBMT (JACIE), Gross National Income (GNI)/capita based on purchasing power parity (PPP) (GNI/cap), donor type, donor-patient sex-match, type of conditioning, stem cell source and T-cell depletion (TCD). Results:Five-year EFS of the whole cohort was 37% (95% Confidence Interval, 33%-42%), Day-100 survival was 90% (88%-92%). Experience of the transplant center was measured by the number of all alloHCTs, and alloHCTs for patients with CLL respectively. The median total number of alloHCTs per center per year was 45 (range 0-169) and the median number of CLL alloHCTs was only 2 per center per year (range 0-19). Greater experience with transplantation of patients with CLL (Hazard Ratio (HR) 0.96 per additional transplant, p=0.002), JACIE accreditation (HR 0.7, p=0.045) and a higher GNI/cap (HR 0.4, 95% CI 0.2-0.96, p=0.04) showed a protective impact on 5-year EFS in the Cox model. In vivo TCD with alemtuzumab (HR 1.5 compared to no TCD, p=0.03) and a female donor for a male patient (HR 1.4 compared to a male donor for a male patient, p=0.02) were the only procedure-related factors significantly associated with EFS. Event-Free Survival after in vivo TCD with Anti-Thymocyte-Globulin or after ex vivo TCD was comparable to EFS without TCD (HR 0.9, 0.7-1.3, p=0.6; HR 0.9, 0.5-1.6, p=0.8). Non-myeloablative conditioning did not have a negative impact on 5-year EFS, and exposed patients to a lower risk of non-relapse mortality. Measured and unmeasured center characteristics did not have a significant impact on 100-day mortality. Even when correcting for patient-, procedure- and center-related characteristics, there was still significant variation in center outcome, expressed by center-specific HRs derived from the frailty models, ranging from 0.6 to 1.2. Their impact is illustrated in a model-based plot for EFS (see Figure) which shows outcomes for three reference patients with the same characteristics who would be transplanted in three centers with the same measured characteristics but with the highest, average and lowest HRs in the dataset. These unexplained center effects likely represent a mixture of differences which could apply to the location of the transplant center, unmeasured characteristics of the patient population transplanted at this center, selection criteria which were not reported and factors determining the success of the transplant procedure which might differ between centers. Conclusion: We have confirmed that both center- and procedure-related factors have a significant impact on the EFS of patients with CLL undergoing alloHCT. Our results may help to interpret outcomes of single or multicenter studies better. Since non-myeloablative conditioning did not have a negative impact on EFS and exposed patients to a lower risk of non-relapse mortality, this approach should be favored for future alloHCT for CLL. Probability of Event-Free Survival up to Five Years Post-HCT for three Reference Patients Contribution: J.S. designed the research and wrote the paper. L.C.d.W conducted the statistical analysis and produced the figure. Figure Figure. Disclosures Schetelig: Sanofi: Honoraria. Gramatzki:Janssen: Other: Travel/Accommodation/Expenses, Research Funding. Dreger:Gilead: Consultancy; Gilead: Speakers Bureau; Janssen: Consultancy; Novartis: Speakers Bureau; Novartis: Consultancy; Roche: Consultancy.

Published
2017

241. Anterior Aortic Plane Systolic Excursion: A Novel Indicator of Transplant-Free Survival in Systemic Light-Chain Amyloidosis

Author

Ochs, Marco M., Riffel, Johannes, Kristen, Arnt V., Hegenbart, Ute, Schönland, Stefan, Hardt, Stefan E., Katus, Hugo A., Mereles, Derliz, and Buss, Sebastian J.

Abstract

Anterior aortic plane systolic excursion (AAPSE) was evaluated in the present pilot study as a novel echocardiographic indicator of transplant-free survival in patients with systemic light-chain amyloidosis.

Published
2024
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242. A novel risk score to predict survival in advanced heart failure due to cardiac amyloidosis.

Author

Kreusser, Michael M., Volz, Martin J., Knop, Benjamin, Ehlermann, Philipp, Schmack, Bastian, Ruhparwar, Arjang, Hegenbart, Ute, Schönland, Stefan O., Katus, Hugo A., and Raake, Philip W.

Abstract

Background: Cardiac amyloidosis, caused by deposition of immunoglobulin light chains (AL) or transthyretin (ATTR), carries a poor prognosis. Established risk scores for amyloidosis may not predict outcomes in those patients who develop advanced heart failure and who are potential candidates for heart transplantation. Here, we aimed to identify predictive parameters for patients with severe heart failure due to amyloidosis. Methods: Out of > 1000 patients with cardiac amyloidosis (AL or ATTR) admitted to our centre between September 1998 and January 2016, a cohort of 120 patients with a complete cardiac assessment at diagnosis, including right heart catheterization, echocardiography and biomarkers, was analysed retrospectively in this study. Primary endpoint was all-cause mortality. We performed univariate and multivariate Cox regression analysis, generated risk scores to predict outcomes in AL and ATTR amyloidosis and compared those to established risk models for amyloidosis. Results: In the Cox multivariate model, high-sensitivity troponin T (hsTnT; hazard ratio (HR) 1.003; confidence interval (CI) 1.001–1.005; p = 0.009) and mean pulmonary artery pressure (HR 1.061; CI 1.024–1.100; p = 0.001) were found to significantly and independently predict outcomes for AL amyloidosis, whereas QRS duration (HR 1.021; CI 1.004–1.039; p = 0.013), hsTnT (HR 1.021; CI 1.006–1.036; p = 0.006) and N-terminal pro-brain natriuretic peptide (HR 1.0003; CI 1.0001–1.0004; p = 0.002) were the best predictors for ATTR amyloidosis. A simple risk score ("HeiRisk") including these parameters for AL and ATTR allowed a more precise risk stratification in our patient population compared to established risk models. Conclusions: Risk stratification for cardiac amyloidosis with the newly developed "HeiRisk" score may be superior to other staging systems for patients with advanced heart failure due to amyloid cardiomyopathy. [ABSTRACT FROM AUTHOR]

Published
2020
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243. Conditioning‐based outcomes after allogeneic transplantation for myeloma following a prior autologous transplant (1991‐2012) on behalf of EBMT CMWP.

Author

Hayden, Patrick J., Iacobelli, Simona, Pérez‐Simón, José Antonio, van Biezen, Anja, Minnema, Monique, Niittyvuopio, Riitta, Schönland, Stefan, Meijer, Ellen, Blaise, Didier, Milpied, Noel, Márquez‐Malaver, Francisco J., Veelken, Joan Hendrik, Maertens, Johan, Michallet, Mauricette, Cammenga, Jörg, N'Guyen, Stephanie, Niederwieser, Dietger, Hunault‐Berger, Mathilde, Bourhis, Jean Henri, and Passweg, Jakob

Subjects
AUTOGRAFTS, TRANSPLANTATION of organs, tissues, etc., PROBABILITY theory, RETROSPECTIVE studies
Abstract

Objectives: The aim of this study was to compare the effect of the intensity of conditioning approaches used in allogeneic transplantation in myeloma—reduced intensity conditioning (RIC), non‐myeloablative (NMA), myeloablative conditioning (MAC) or Auto‐AlloHCT—on outcomes in patients who had had a prior autologous transplant. Methods: A retrospective analysis of the EBMT database (1991‐2012) was performed. Results: A total of 344 patients aged between 40 and 60 years at the time of alloHCT were identified: 169 RIC, 69 NMA, 65 MAC and 41 Auto‐Allo transplants. At a median follow‐up of 54 months, the probabilities of overall survival (OS) at 5 years were 39% (95% CI 31%‐47%), 45% (95% CI 32%‐57%), 19% (95% CI 6%‐32%) and 34% (95% CI 17%‐51%), respectively. Status at allogeneic HCT other than CR or PR conferred a 70% higher risk of death and a 40% higher risk of relapse. OS was markedly lower in the MAC group (P =.004). MAC alloHCT was associated with a higher risk of death than RIC alloHCT until 2002 (HR = 4.1, P <.001) but not after 2002 (HR = 1.2, P =.276). Conclusion: From 1991 to 2002, MAC was associated with poorer OS. Between 2003 and 2012, there were no significant differences in outcomes based on these different approaches. [ABSTRACT FROM AUTHOR]

Published
2020
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244. Carpal tunnel syndrome and spinal canal stenosis: harbingers of transthyretin amyloid cardiomyopathy?

Author

aus dem Siepen, Fabian, Hein, Selina, Prestel, Sofie, Baumgärtner, Christian, Schönland, Stefan, Hegenbart, Ute, Röcken, Christoph, Katus, Hugo A., and Kristen, Arnt V.

Abstract

Background: Carpal tunnel syndrome (CTS) and spinal canal stenosis can be frequently observed in the medical history of patients with transthyretin amyloidosis (ATTR), both in the hereditary (mt-ATTR) and wild-type (wt-ATTR) form. The aim of this retrospective single-center analysis was to determine the prevalence of these findings, delay to diagnosis of systemic amyloidosis and the prognostic value in a large cohort of patients with wt-ATTR and mt-ATTR amyloidosis. Methods: Medical records of 253 patients diagnosed with wt-ATTR, 136 patients with mt-ATTR and 77 asymptomatic gene carriers were screened for history of CTS and spinal canal stenosis and laboratory analysis, electrocardiography and echocardiographic results, respectively. Clinical follow-up was performed by phone assessment. Results: History of CTS was present in 77 patients (56%) with mt-ATTR, in 152 patients (60%) with wt-ATTR and even in 10 of the asymptomatic gene carriers (13%). Latency between carpal tunnel surgery and first diagnosis of systemic amyloidosis was significantly longer in wt-ATTR compared to mt-ATTR (117 ± 179 months vs. 66 ± 73 months; p = 0.02). In total, 36 patients (14%) with wt-ATTR and 7 patients (5%) with mt-ATTR had a history of clinically significant spinal canal stenosis. In the subgroup of mt-ATTR, patients with CTS had thicker IVS (19 ± 5 mm vs. 16 ± 5 mm, p < 0.05), higher LV mass index (225 ± 78 g vs. 193 ± 98 g, p < 0.05), lower Karnofsky index (78 ± 15% vs. 83 ± 17%, p < 0.05), and lower mitral annular plane systolic excursion (MAPSE; 9 ± 4 mm vs. 11 ± 5 mm, p < 0.05) compared to patients without CTS, whereas in wt-ATTR no significant differences could be observed. No significant difference in survival was observed between patients with and without CTS (wt-ATTR: 67 vs. 63 months, p = 0.45; mt-ATTR: 74 vs. 63 months, p = 0.60). A combination of CTS and spinal stenosis was present in 32 wt-ATTR patients (12%) and 3 mt-ATTR patients (2.2%). Conclusions: The prevalence of CTS is high and the latency between CTS surgery and diagnosis of amyloidosis is long among patients with wt-ATTR and mt-ATTR. CTS might be predictive for future occurrence of systemic (predominantly cardiac) ATTR amyloidosis. [ABSTRACT FROM AUTHOR]

Published
2019
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245. Reduced intensity conditioning regimens including alkylating chemotherapy do not alter survival outcomes after allogeneic hematopoietic cell transplantation in chronic lymphocytic leukemia compared to low-intensity non-myeloablative conditioning.

Author

Andersen, Niels Smedegaard, Bornhäuser, Martin, Gramatzki, Martin, Dreger, Peter, Vitek, Antonin, Karas, Michal, Michallet, Mauricette, Moreno, Carol, van Gelder, Michel, Henseler, Anja, de Wreede, Liesbeth C., Schönland, Stefan, Kröger, Nicolaus, and Schetelig, Johannes

Subjects
CHRONIC lymphocytic leukemia, CELL transplantation, HEMATOPOIETIC stem cell transplantation
Abstract

Purpose: The optimal dose intensity for conditioning prior to allogeneic hematopoietic stem cell transplantation (alloHSCT) for chronic lymphocytic leukemia (CLL) is unknown. Methods: We retrospectively compared outcomes of patients who received a first alloHCST after non-myeloablative (NMA) and reduced intensity conditioning (RIC). Data of 432 patients with a median age of 55 years were included, of which 86 patients underwent NMA and 346 RIC. Results: The median follow-up after alloHSCT was 4.3 years. Compared to the RIC group, more NMA patients had purine-analog-sensitive disease, were in complete remission and received matched related donor transplantation. After RIC, the probabilities for 5-year OS, EFS, CIR, and NRM were 46%, 38%, 28%, and 35% and after NMA the respective probabilities were 52%, 43%, 25%, and 32%. In multivariate analysis, remission status prior to conditioning but not RIC versus NMA conditioning had a significant impact on CIR, EFS, and OS. Conclusion: Presumed higher anti-leukemic activity of RIC versus NMA conditioning did not translate into better outcomes after alloHSCT, but better remission status prior to conditioning did. Effective pathway inhibitor-based salvage therapies combined with NMA conditioning might thus represent the most attractive contemporary approach for alloHSCT for patients with CLL. [ABSTRACT FROM AUTHOR]

Published
2019
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248. CD7 is expressed on a subset of normal CD34-positive myeloid precursors

Author

Kriegsmann, Katharina, primary, Löffler, Harald, additional, Eckstein, Volker, additional, Schulz, Renate, additional, Kräker, Sandra, additional, Braun, Ute, additional, Luft, Thomas, additional, Hegenbart, Ute, additional, Schönland, Stefan, additional, Dreger, Peter, additional, Krämer, Alwin, additional, Ho, Anthony D., additional, Müller-Tidow, Carsten, additional, and Hundemer, Michael, additional

Published
2018
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249. Outcome of a Salvage Third Autologous Stem Cell Transplantation in Multiple Myeloma

Author

Garderet, Laurent, primary, Iacobelli, Simona, additional, Koster, Linda, additional, Goldschmidt, Hartmut, additional, Johansson, Jan-Erik, additional, Bourhis, Jean Henri, additional, Krejci, Marta, additional, Leleu, Xavier, additional, Potter, Michael, additional, Blaise, Didier, additional, Koenecke, Christian, additional, Peschel, Christian, additional, Radocha, Jakub, additional, Metzner, Bernd, additional, Lenain, Pascal, additional, Schäfer-Eckart, Kerstin, additional, Pohlreich, David, additional, Grasso, Mariella, additional, Caillot, Denis, additional, Einsele, Herman, additional, Ladetto, Marco, additional, Schönland, Stefan, additional, and Kröger, Nicolaus, additional

Published
2018
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250. Subcutaneous daratumumab (DARA SC) plus cyclophosphamide, bortezomib, and dexamethasone (CyBorD) in patients (Pts) with newly diagnosed amyloid light chain (AL) amyloidosis: Safety run-in results of andromeda.

Author

Comenzo, Ray, primary, Kastritis, Efstathios, additional, Maurer, Mathew, additional, Zonder, Jeffrey A., additional, Minnema, Monique, additional, Schönland, Stefan, additional, Wechalekar, Ashutosh, additional, Palladini, Giovanni, additional, Qin, Xiang, additional, Vasey, Sandra Y., additional, Khan, Imran, additional, Schecter, Jordan Mark, additional, and Merlini, Giampaolo, additional

Published
2018
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