Search

Your search keyword '"Faraci, Maura"' showing total 16 results
Start Over Author "Faraci, Maura" Database OpenAIRE
16 results on '"Faraci, Maura"'

1. Progressive Multifocal Leukoencephalopathy (PML) after HCT - A Retrospective Study of Infectious Diseases working Party EBMT

Author

Dybko, Jaroslaw, Gil, Lidia, Wendel, Lotus, Knelange, Nina, Kroeger, Nicoalus, Hayat, Amjad, Liga, Maria, Blijlevens, Nicole, Bazarbachi, Ali, Faraci, Maura, Arnold, Ganser, Steinhardt, Maximilian, Gruhn, Bernd, Stamouli, Maria, Villar, Sara, Pass-weg, Jakob, Schots, Rik, Bernasconi, Paolo, Zucker-man, Tsila, Mikulska, Malgorzata, Styczynski, Jan, Faculty of Law and Criminology, Clinical sciences, and Hematology

Published
2020

2. Additional file 1: of Early gut microbiota signature of aGvHD in children given allogeneic hematopoietic cell transplantation for hematological disorders

Author

Biagi, Elena, Zama, Daniele, Rampelli, Simone, Turroni, Silvia, Brigidi, Patrizia, Consolandi, Clarissa, Severgnini, Marco, Picotti, Eleonora, Gasperini, Pietro, Merli, Pietro, Decembrino, Nunzia, Zecca, Marco, Cesaro, Simone, Faraci, Maura, Prete, Arcangelo, Locatelli, Franco, Pession, Andrea, Candela, Marco, and Masetti, Riccardo

Subjects
surgical procedures, operative, integumentary system, immune system diseases, hemic and lymphatic diseases
Abstract

Figure S1. PCoA based on Bray-Curtis distances of genus-level relative abundance profiles of the samples sequenced in the present study using Illumina HiSeq and those from the study of Biagi et al. [7] sequenced by Roche 454 pyrosequencing. Figure S2. PCoA based on Bray-Curtis distances of genus-level relative abundance profiles of samples taken from patients aged 12 years, before, 12–28 days after, and > 30 days after HSCT. Figure S3. PCoA based on Bray-Curtis distances of genus-level relative abundance profiles of samples taken from patients hospitalized in the four centers before, 12–28 days after, and > 30 days after HSCT. Figure S4. Box and whiskers distribution of the relative abundance (%) of OTUs assigned to the genera Enterococcus, Citrobacter and Erwinia, in samples grouped as pre-HSCT, engraftment and post-HSCT, for the four enrollment centers. Figure S5. Box and whiskers distribution of the Simpson diversity index calculated for the genus-level microbiota profiles in samples taken before HSCT from subjects who did not develop aGvHD, who developed aGvHD (I-II grade) at a skin level, and who developed gastrointestinal aGvHD (II-IV grade). Figure S6. Box and whiskers distribution of the Bray-Curtis distances calculated using the genus-level gut microbiota profiles of samples taken before HSCT from subjects who did not develop aGvHD, who developed aGvHD (I-II grade) at a skin level, and who developed gastrointestinal aGvHD (II-IV grade). Figure S7. PCoA based on Bray-Curtis distances of genus-level relative abundance profiles of samples taken > 30 days after HSCT from patients who did not develop aGvHD, who developed aGvHD (I-II grade) at a skin level, and who developed gastrointestinal aGvHD (II-IV grade). (PDF 305 kb)

Published
2019
Full Text
View/download PDF

3. Additional file 2: of Early gut microbiota signature of aGvHD in children given allogeneic hematopoietic cell transplantation for hematological disorders

Author

Biagi, Elena, Zama, Daniele, Rampelli, Simone, Turroni, Silvia, Brigidi, Patrizia, Consolandi, Clarissa, Severgnini, Marco, Picotti, Eleonora, Gasperini, Pietro, Merli, Pietro, Decembrino, Nunzia, Zecca, Marco, Cesaro, Simone, Faraci, Maura, Prete, Arcangelo, Locatelli, Franco, Pession, Andrea, Candela, Marco, and Masetti, Riccardo

Subjects
surgical procedures, operative, integumentary system, immune system diseases, hemic and lymphatic diseases
Abstract

Tables S1. Average relative abundances in pre-HSCT and engraftment samples, of the main discriminant genera between the two time points in subjects who did not develop aGvHD (non-aGvHD), who developed aGvHD (I-II grade) at a skin level (Skin aGvHD), and who developed gastrointestinal aGvHD (II-IV grade) (Gut aGvHD) (Wilcoxon test, P ≤ 0.05, in bold, in at least one group of subjects). P values between 0.05 and 0.1 are reported in italics, whereas P values > 0.1 are reported as “ns”, not significant. (PDF 257 kb)

Published
2019
Full Text
View/download PDF

4. Incidence, Risk Factors and Outcome of Pre-engraftment Gram-Negative Bacteremia After Allogeneic and Autologous Hematopoietic Stem Cell Transplantation: An Italian Prospective Multicenter Survey

Author

Girmenia, Corrado, Bertaina, Alice, Piciocchi, Alfonso, Perruccio, Katia, Algarotti, Alessandra, Busca, Alessandro, Cattaneo, Chiara, Raiola, Anna Maria, Guidi, Stefano, Iori, Anna Paola, Candoni, Anna, Irrera, Giuseppe, Milone, Giuseppe, Marcacci, Giampaolo, Scimè, Rosanna, Musso, Maurizio, Cudillo, Laura, Sica, Simona, Castagna, Luca, Corradini, Paolo, Marchesi, Francesco, Pastore, Domenico, Alessandrino, Emilio Paolo, Annaloro, Claudio, Ciceri, Fabio, Santarone, Stella, Nassi, Luca, Farina, Claudio, Viscoli, Claudio, Rossolini, Gian Maria, Bonifazi, Francesca, Rambaldi, Alessandro, Capria, Saveria, Bertaina, A., Mastronuzzi, Angela, Pagliara, Daria, Bernaschi, Paola, Amico, Lucia, Carotti, Alessandra, Mencacci, Antonella, Busca, A., Bruno, Benedetto, Costa, Cristina, Passi, Angela, Ravizzola, Giuseppe, Angelucci, Emanuele, Marchese, Anna, Pecile, Patrizia, Candoni, A., Ventura, Giovanna, Fanin, Renato, Scarparo, Claudio, Barbaro, Angelo, Milone, G., Leotta, Salvatore, Marchese, Anna Elisa, Marcacci, G., Becchimanzi, Cristina, Donnarumma, Daniela, Tringali, Stefania, Baldi, Maria Teresa, Scalone, Renato, Cudillo, L., Picardi, Alessandra, Arcese, William, Fontana, Carla, Sica, S., Giammarco, Sabrina, Spanu, Teresa, Castagna, L., Crocchiolo, Roberto, Casari, Erminia, Mussetti, Alberto, Conte, Eutilia, Ensoli, Fabrizio, Miragliotta, Giuseppe, Marone, Piero, Arghittu, Milena, Greco, Raffaella, Forcina, Alessandra, Chichero, Paola, Santarone, S., Di Bartolomeo, Paolo, Fazii, Paolo, Kroumova, Vesselina, Decembrino, Nunzia, Zecca, Marco, Pisapia, Giovanni, Palazzo, Giulia, Lanino, Edoardo, Faraci, Maura, Castagnola, Elio, Bandettini, Roberto, Pastano, Rocco, Sammassimo, Simona, Passerini, Rita, Stefani, Piero Maria, Gherlinzoni, Filippo, Rigoli, Roberto, Prezioso, Lucia, Cambò, Benedetta, Calderaro, Adriana, Carella, Angelo Michele, Cascavilla, Nicola, Labonia, Maria Teresa, Celeghini, Ivana, Mordini, Nicola, Piana, Federica, Vacca, Adriana, Sanna, Marco, Podda, Giovanni, Corsetti, Maria Teresa, Rocchetti, Andrea, Cilloni, Daniela, De Gobbi, Marco, Bianco, Ornella, Fagioli, Franca, Carraro, Francesca, De Intinis, Gianfranco, Severino, Alessandro, Proia, Anna, Parisi, Gabriella, Vallisa, Daniele, Confalonieri, Massimo, Russo, Domenico, Malagola, Michele, Galieni, Piero, Falcioni, Sadia, Travaglini, Valeria, Raimondi, Roberto, Borghero, Carlo, Pavan, Giacomina, Prete, Arcangelo, Belotti, Tamara, Ambretti, Simone, Imola, Manuela, Mianulli, Anna Maria, Pedna, Maria Federica, Cesaro, Simone, Lo Cascio, Giuliana, Ferrari, Antonella, Piedimonte, Monica, Santino, Iolanda, Calandrelli, Monica, Olivieri, Attilio, Orecchioni, Francesca, Mirabile, Milena, Centurioni, Riccardo, Gironacci, Luciana, Caravelli, Daniela, Gallo, Susanna, De Filippi, Marco, Cupelli, Luca, Dentamaro, Teresa, Falco, Silvana, Eugenio, Ospedale S, Marotta, Serena, Risitano, Antonio, Lula, Dora, Musto, Pellegrino, Pietrantuono, Giuseppe, Traficante, Antonio, Cerchiara, Elisabetta, Tirindelli, Maria Cristina, Dicuonzo, Giordano, Chierichini, Anna, Anaclerico, Barbara, Placanica, Paola, Girmenia, C, Bertaina, A, Piciocchi, A, Perruccio, K, Algarotti, A, Busca, A, Cattaneo, C, Raiola, Am, Guidi, S, Iori, Ap, Candoni, A, Irrera, G, Milone, G, Marcacci, G, Scimè, R, Musso, M, Cudillo, L, Sica, S, Castagna, L, Corradini, P, Marchesi, F, Pastore, D, Alessandrino, Ep, Annaloro, C, Ciceri, F, Santarone, S, Nassi, L, Farina, C, Viscoli, C, Rossolini, Gm, Bonifazi, F, Rambaldi, A, for the Gruppo Italiano Trapianto di Midollo Osseo, (GITMO), Associazione Microbiologi Clinici Italiani, (AMCLI), Girmenia, Corrado, Bertaina, Alice, Piciocchi, Alfonso, Perruccio, Katia, Algarotti, Alessandra, Busca, Alessandro, Cattaneo, Chiara, Raiola, Anna Maria, Guidi, Stefano, Iori, Anna Paola, Candoni, Anna, Irrera, Giuseppe, Milone, G., Marcacci, Giampaolo, Scimè, Rosanna, Musso, Maurizio, Cudillo, Laura, Sica, Simona, Castagna, Luca, Corradini, Paolo, Marchesi, Francesco, Pastore, Domenico, Alessandrino, Emilio Paolo, Annaloro, Claudio, Ciceri, Fabio, Santarone, Stella, Nassi, Luca, Farina, Claudio, Viscoli, Claudio, Rossolini, Gian Maria, Bonifazi, Francesca, Rambaldi, Alessandro, Capria, Saveria, Bertaina, A., Mastronuzzi, Angela, Pagliara, Daria, Bernaschi, Paola, Amico, Lucia, Carotti, Alessandra, Mencacci, Antonella, Busca, A., Bruno, Benedetto, Costa, Cristina, Passi, Angela, Ravizzola, Giuseppe, Angelucci, Emanuele, Marchese, Anna, Pecile, Patrizia, Candoni, A., Ventura, Giovanna, Fanin, Renato, Scarparo, Claudio, Barbaro, Angelo, Leotta, Salvatore, Marchese, Anna Elisa, Marcacci, G., Becchimanzi, Cristina, Donnarumma, Daniela, Tringali, Stefania, Baldi, Maria Teresa, Scalone, Renato, Cudillo, L., Picardi, Alessandra, Arcese, William, Fontana, Carla, Sica, S., Giammarco, Sabrina, Spanu, Teresa, Castagna, L., Crocchiolo, Roberto, Casari, Erminia, Mussetti, Alberto, Conte, Eutilia, Ensoli, Fabrizio, Miragliotta, Giuseppe, Marone, Piero, Arghittu, Milena, Greco, Raffaella, Forcina, Alessandra, Chichero, Paola, Santarone, S., Di Bartolomeo, Paolo, Fazii, Paolo, Kroumova, Vesselina, Decembrino, Nunzia, Zecca, Marco, Pisapia, Giovanni, Palazzo, Giulia, Lanino, Edoardo, Faraci, Maura, Castagnola, Elio, Bandettini, Roberto, Pastano, Rocco, Sammassimo, Simona, Passerini, Rita, Stefani, Piero Maria, Gherlinzoni, Filippo, Rigoli, Roberto, Prezioso, Lucia, Cambò, Benedetta, Calderaro, Adriana, Carella, Angelo Michele, Cascavilla, Nicola, Labonia, Maria Teresa, Celeghini, Ivana, Mordini, Nicola, Piana, Federica, Vacca, Adriana, Sanna, Marco, Podda, Giovanni, Corsetti, Maria Teresa, Rocchetti, Andrea, Cilloni, Daniela, De Gobbi, Marco, Bianco, Ornella, Fagioli, Franca, Carraro, Francesca, De Intinis, Gianfranco, Severino, Alessandro, Proia, Anna, Parisi, Gabriella, Vallisa, Daniele, Confalonieri, Massimo, Russo, Domenico, Malagola, Michele, Galieni, Piero, Falcioni, Sadia, Travaglini, Valeria, Raimondi, Roberto, Borghero, Carlo, Pavan, Giacomina, Prete, Arcangelo, Belotti, Tamara, Ambretti, Simone, Imola, Manuela, Mianulli, Anna Maria, Pedna, Maria Federica, Cesaro, Simone, Lo Cascio, Giuliana, Ferrari, Antonella, Piedimonte, Monica, Santino, Iolanda, Calandrelli, Monica, Olivieri, Attilio, Orecchioni, Francesca, Mirabile, Milena, Centurioni, Riccardo, Gironacci, Luciana, Caravelli, Daniela, Gallo, Susanna, De Filippi, Marco, Cupelli, Luca, Dentamaro, Teresa, Falco, Silvana, Eugenio, Ospedale S, Marotta, Serena, Risitano, Antonio, Lula, Dora, Musto, Pellegrino, Pietrantuono, Giuseppe, Traficante, Antonio, Cerchiara, Elisabetta, Tirindelli, Maria Cristina, Dicuonzo, Giordano, Chierichini, Anna, Anaclerico, Barbara, and Placanica, Paola

Subjects
0301 basic medicine, Male, Transplantation Conditioning, Drug Resistance, Bacteremia, 0302 clinical medicine, epidemiology, Gram negative bacteremia, multidrug resistance, stem cell transplant, survival, Risk Factors, Drug Resistance, Multiple, Bacterial, Epidemiology, Medicine, Age Factor, Prospective Studies, Prospective cohort study, Child, Transplantation, Homologou, education.field_of_study, Incidence (epidemiology), Incidence, Bacterial, Age Factors, Hematopoietic Stem Cell Transplantation, Middle Aged, Transplantation, Autologou, surgical procedures, operative, Infectious Diseases, Italy, Child, Preschool, Female, medicine.symptom, Multiple, Autologous, Human, Homologous, Microbiology (medical), Adult, medicine.medical_specialty, Adolescent, 030106 microbiology, Population, Transplantation, Autologous, 03 medical and health sciences, Young Adult, Internal medicine, Gram-Negative Bacteria, Gram-Negative Bacterial Infection, Escherichia coli, Humans, Transplantation, Homologous, Preschool, education, Aged, Gram-Negative Bacterial Infections, Infant, Transplantation, business.industry, Risk Factor, medicine.disease, Prospective Studie, Carriage, Otitis, business, Settore MED/15 - Malattie del Sangue, 030215 immunology
Abstract

Background Gram-negative bacteremia (GNB) is a major cause of illness and death after hematopoietic stem cell transplantation (HSCT), and updated epidemiological investigation is advisable. Methods We prospectively evaluated the epidemiology of pre-engraftment GNB in 1118 allogeneic HSCTs (allo-HSCTs) and 1625 autologous HSCTs (auto-HSCTs) among 54 transplant centers during 2014 (SIGNB-GITMO-AMCLI study). Using logistic regression methods. we identified risk factors for GNB and evaluated the impact of GNB on the 4-month overall-survival after transplant. Results The cumulative incidence of pre-engraftment GNB was 17.3% in allo-HSCT and 9% in auto-HSCT. Escherichia coli, Klebsiella pneumoniae, and Pseudomonas aeruginosa were the most common isolates. By multivariate analysis, variables associated with GNB were a diagnosis of acute leukemia, a transplant from a HLA-mismatched donor and from cord blood, older age, and duration of severe neutropenia in allo-HSCT, and a diagnosis of lymphoma, older age, and no antibacterial prophylaxis in auto-HSCT. A pretransplant infection by a resistant pathogen was significantly associated with an increased risk of posttransplant infection by the same microorganism in allo-HSCT. Colonization by resistant gram-negative bacteria was significantly associated with an increased rate of infection by the same pathogen in both transplant procedures. GNB was independently associated with increased mortality at 4 months both in allo-HSCT (hazard ratio, 2.13; 95% confidence interval, 1.45–3.13; P Conclusions Pre-engraftment GNB is an independent factor associated with increased mortality rate at 4 months after auto-HSCT and allo-HSCT. Previous infectious history and colonization monitoring represent major indicators of GNB. Clinical Trials registration NCT02088840.

Published
2017

7. Cardiac and pulmonary late effects do not negatively influence performance status and non-relapse mortality of children surviving five yr after autologous hematopoietic cell transplantation

Author

Uderzo, Cornelio, Pillon, Marta, Tridello, Gloria, Dini, Giorgio, Urban, Christian, Corti, Paola, Zintl, Felix, Fagioli, Franca, Messina, Chiara, Verdeguer, Amparo, Faraci, Maura, Fedeli, Sara, Tana, Francesco, Tichelli, André, Passweg, Jakob, and Rovelli, Attilio

Subjects
ddc:616, Male, Time Factors, Adolescent, Incidence, Hematopoietic Stem Cell Transplantation/adverse effects/methods, Infant, Lung/physiology, Heart/physiology, Respiratory Function Tests, Pediatrics/methods, Treatment Outcome, Child, Preschool, Humans, Female, Prospective Studies, Child
Abstract

The current prospective study dealt with clinical outcome associated with pulmonary and cardiac late effects of AuHCT in children with malignancies. We prospectively evaluated 58 children, utilizing pulmonary function tests and cardiac shortening fraction, performed in pre-AuHCT phase and then annually. The overall five-yr survival was 68%. The five-yr cumulative incidence of lung and cardiac function impairment in survivors was 21% in both cases. None of the patients presented with restrictive or obstructive pulmonary pathology at the last follow-up and performance status for all survivors, ranged from 90% to 100%. The cumulative incidence of non-relapse mortality was 12.6% (range 6.3-25.3%), whereas relapse mortality was 19.7% (range 11.6-33.5). In conclusion, our study shows no significant deterioration in post-AuHCT pulmonary and cardiac function and in particular, no negative impact of lung and heart late effects on performance status and non-relapse mortality.

Published
2009

8. Occult hepatitis B virus infection: a case of reactivation in a patient receiving immunosuppressive treatment for allogeneic bone marrow transplantation

Author

Giudice, Cinzia Lo, Martinengo, Marina, Pietrasanta, Paolo, Bocciardo, Laura, Malavasi, Cristina, Rastelli, Simona, Faraci, Maura, and Gino, Tripodi

Subjects
Hepatitis B virus, Immunocompromised Host, Hepatitis B Surface Antigens, Hepatitis B, Chronic, Transplantation Conditioning, Adolescent, DNA, Viral, Humans, Case Report, Bone Marrow Transplantation
Published
2008

14. Outcome of haematopoietic stem cell transplantation in dyskeratosis congenita

Author

Julián Sevilla, Paul Veys, E T Korthof, Régis Peffault de Latour, Dorine Bresters, Tracey A. O'Brien, Marco Zecca, Maura Faraci, Simona Iacobelli, Jean Hugues Dalle, Antonio M. Risitano, Luca Arcuri, Maurizio Miano, Francesca Fioredda, Ardeshir Ghavamzadeh, Reuven Or, Miguel Angel Diaz, Brune Mats, E V Skorobogatova, Josue de la Fuente, Cora Knol, Petr Sedlacek, Franca Fagioli, Cristina Díaz de Heredia, Ayami Yoshimi, Michael Maschan, Jakub Tolar, Owen P. Smith, Carlo Dufour, Mahmoud Aljurf, Maria Teresa Van Lint, Alain Fisher, Anja van Biezen, Fioredda, Francesca, Iacobelli, Simona, Korthof, Elisabeth T., Knol, Cora, van Biezen, Anja, Bresters, Dorine, Veys, Paul, Yoshimi, Ayami, Fagioli, Franca, Mats, Brune, Zecca, Marco, Faraci, Maura, Miano, Maurizio, Arcuri, Luca, Maschan, Michael, O'Brien, Tracey, Diaz, Miguel A., Sevilla, Julian, Smith, Owen, Peffault de Latour, Regi, de la Fuente, Josue, Or, Reuven, Van Lint, Maria T., Tolar, Jakub, Aljurf, Mahmoud, Fisher, Alain, Skorobogatova, Elena V., Diaz de Heredia, Cristina, Risitano, Antonio, Dalle, Jean-Hugue, Sedláček, Petr, Ghavamzadeh, Ardeshir, and Dufour, Carlo

Subjects
Adult, Male, Oncology, medicine.medical_specialty, Transplantation Conditioning, Pulmonary Fibrosis, haematopoietic stem cell transplantation, Graft vs Host Disease, Disease, dyskeratosis congenita, Settore MED/01 - Statistica Medica, Young Adult, bone marrow failure, Hematology, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, Humans, Bone Marrow Diseases, business.industry, Age Factors, Hematopoietic Stem Cell Transplantation, Bone marrow failure, medicine.disease, Survival Analysis, Tissue Donors, Transplantation, Haematopoiesis, Treatment Outcome, surgical procedures, operative, medicine.anatomical_structure, 030220 oncology & carcinogenesis, Female, Bone marrow, Stem cell, business, Dyskeratosis congenita, 030215 immunology
Abstract

Dyskeratosis congenita (DC) is a genetic multisystem disorder with frequent involvement of the bone marrow. Haematopoietic stem cell transplantation (HSCT) is the only definitive cure to restore haematopoiesis, even though it cannot correct other organ dysfunctions. We collected data on the outcome of HSCT in the largest cohort of DC (n = 94) patients ever studied. Overall survival (OS) and event-free survival (EFS) at 3 years after HSCT were 66% and 62%, respectively. Multivariate analysis showed better outcomes in patients aged less than 20 years and in patients transplanted from a matched, rather than a mismatched, donor. OS and EFS curves tended to decline over time. Early lethal events were infections, whereas organ damage and secondary malignancies appeared afterwards, even a decade after HSCT. A non-myeloablative conditioning regimen appeared to be most advisable. Organ impairment present before HSCT seemed to favour the development of chronic graft-versus-host disease and T-B immune deficiency appeared to enhance pulmonary fibrosis. According to the present data, HSCT in DC is indicated in cases of progressive marrow failure, whereas in patients with pre-existing organ damage, this should be carefully evaluated. Further efforts to investigate treatment alternatives to HSCT should be encouraged.

Published
2018

15. Early gut microbiota signature of aGvHD in children given allogeneic hematopoietic cell transplantation for hematological disorders

Author

Silvia Turroni, Simone Rampelli, Riccardo Masetti, Maura Faraci, Eleonora Picotti, Clarissa Consolandi, Elena Biagi, Patrizia Brigidi, Arcangelo Prete, Marco Candela, Marco Severgnini, Pietro Gasperini, Nunzia Decembrino, Pietro Merli, Simone Cesaro, Marco Zecca, Franco Locatelli, Andrea Pession, Daniele Zama, Biagi, Elena, Zama, Daniele, Rampelli, Simone, Turroni, Silvia, Brigidi, Patrizia, Consolandi, Clarissa, Severgnini, Marco, Picotti, Eleonora, Gasperini, Pietro, Merli, Pietro, Decembrino, Nunzia, Zecca, Marco, Cesaro, Simone, Faraci, Maura, Prete, Arcangelo, Locatelli, Franco, Pession, Andrea, Candela, Marco, and Masetti, Riccardo

Subjects
Male, 0301 basic medicine, Hematological disorders, Time Factors, medicine.medical_treatment, Pediatric patients, Graft vs Host Disease, Hematopoietic stem cell transplantation, Disease, Gut flora, Pediatric patient, 0302 clinical medicine, immune system diseases, hemic and lymphatic diseases, Medicine, 16S rRNA gene sequencing, Genetics (clinical), biology, Biodiversity, surgical procedures, operative, Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA, 030220 oncology & carcinogenesis, Acute Disease, Female, Research Article, Adult, lcsh:Internal medicine, lcsh:QH426-470, Gut microbiota Hematopoietic stem cell transplantation Acute graft-versus-host disease Alloreactivity 16S rRNA gene sequencing Pediatric patients, Acute graft-versus-host disease, Alloreactivity, Gut microbiota, 03 medical and health sciences, Therapeutic approach, Genetic, Genetics, Humans, Transplantation, Homologous, lcsh:RC31-1245, Hematopoietic cell, business.industry, Fecal bacteriotherapy, biology.organism_classification, Hematologic Diseases, Gastrointestinal Microbiome, Transplantation, lcsh:Genetics, 030104 developmental biology, Immunology, business
Abstract

Background The onset of acute Graft-versus-Host Disease (aGvHD) has been correlated with the gut microbiota (GM) composition, but experimental observations are still few, mainly involving cohorts of adult patients. In the current scenario where fecal microbiota transplantation has been used as a pioneer therapeutic approach to treat steroid-refractory aGvHD, there is an urgent need to expand existing observational studies of the GM dynamics in Hematopoietic Stem Cell Transplantation (HSCT). Aim of the present study is to explore the GM trajectory in 36 pediatric HSCT recipients in relation to aGvHD onset. Methods Thirty-six pediatric patients, from four transplantation centers, undergoing HSCT were enrolled in the study. Stools were collected at three time points: before HSCT, at time of engraftment and > 30 days following HSCT. Changes in the GM phylogenetic structure were studied by 16S rRNA gene Illumina sequencing and phylogenetic assignation. Results Children developing gut aGvHD had a dysbiotic GM layout before HSCT occurred. This putative aGvHD-predisposing ecosystem state was characterized by (i) reduced diversity, (ii) lower Blautia content, (iii) increase in Fusobacterium abundance. At time of engraftment, the GM structure underwent a deep rearrangement in all patients but, regardless of the occurrence of aGvHD and its treatment, it reacquired a eubiotic configuration from day 30. Conclusions We found a specific GM signature before HSCT predictive of subsequent gut aGvHD occurrence. Our data may open the way to a GM-based stratification of the risk of developing aGvHD in children undergoing HSCT, potentially useful also to identify patients benefiting from prophylactic fecal transplantation. Electronic supplementary material The online version of this article (10.1186/s12920-019-0494-7) contains supplementary material, which is available to authorized users.

Published
2019

16. Extended clinical and immunological phenotype and transplant outcome in CD27 and CD70 deficiency

Author

Emily S.J. Edwards, Safa Baris, Asghar Aghamohammadi, Hubert Kogler, Emma Gostick, Baran Erman, Geetha Rao, Ekrem Unal, Musa Karakukcu, Funda Erol Cipe, Kaan Boztug, Michael Paulussen, Sule Haskologlu, Sevgi Köstel Bal, Renate Krüger, Bénédicte Neven, David Price, Ruy Perez Becker, Joris M. van Montfrans, Stuart G. Tangye, Sylvain Latour, Tim Niehues, Elif Karakoc-Aydiner, Maura Faraci, Fabian Hauck, Dagmar Berghuis, Elisabeth Salzer, Roland Meisel, Theresa Cole, Yu Zhang, Arjan C. Lankester, Raúl Jiménez Heredia, Austen Worth, Ayse Metin, Emma C. Morris, Tooba Momen, Lennart Hammarström, Bethany Pillay, Claudia Gonzaga-Jauregui, Figen Dogu, Andy I. M. Hoepelman, Ebru Yilmaz, Cindy S. Ma, Sharon Choo, Horst von Bernuth, Ahmet Ozen, Dirk Holzinger, Helen C. Su, Nima Rezaei, Pieter L. A. Fraaij, Samaneh Zoghi, Andrew J. Oler, Aditya Gupta, Candan Islamoglu, Marco Gattorno, Sujal Ghosh, Leo Kager, Ebmt, Prasad T. Oommen, Aydan Ikinciogullari, Gregor Dückers, Inci Ilhan, Michael J. Lenardo, Hassan Abolhassani, Serdar Ceylaner, Qiang Pan-Hammarström, Siobhan O. Burns, Kubra Baskin, E. Graham Davies, Imagine - Institut des maladies génétiques (IHU) (Imagine - U1163), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP), Pediatrics, Virology, Ghosh, Sujal, Bal, Sevgi Koestel, Edwards, Emily S. J., Pillay, Bethany, Heredia, Raul Jimenez, Cipe, Funda Erol, Rao, Geetha, Salzer, Elisabeth, Zoghi, Samaneh, Abolhassani, Hassan, Momen, Tooba, Gostick, Emma, Price, David A., Zhang, Yu, Oler, Andrew J., Gonzaga-Jauregui, Claudia, Erman, Baran, Metin, Ayse, Ilhan, Inci, Haskologlu, Sule, Islamoglu, Candan, Baskin, Kubra, Ceylaner, Serdar, Yilmaz, Ebru, Unal, Ekrem, Karakukcu, Musa, Berghuis, Dagmar, Cole, Theresa, Gupta, Aditya K., Hauck, Fabian, Kogler, Hubert, Hoepelman, Andy I. M., Baris, Safa, Karakoc-Aydiner, Elif, Ozen, Ahmet, Kager, Leo, Holzinger, Dirk, Paulussen, Michael, Krueger, Renate, Meisel, Roland, Oommen, Prasad T., Morris, Emma, Neven, Benedicte, Worth, Austen, van Montfrans, Joris, Fraaij, Pieter L. A., Choo, Sharon, Dogu, Figen, Davies, E. Graham, Burns, Siobhan, Duckers, Gregor, Becker, Ruy Perez, von Bernuth, Horst, Latour, Sylvain, Faraci, Maura, Gattorno, Marco, Su, Helen C., Pan-Hammarstroem, Qiang, Hammarstroem, Lennart, Lenardo, Michael J., Ma, Cindy S., Niehues, Tim, Aghamohammadi, Asghar, Rezaei, Nima, Ikinciogullari, Aydan, Tangye, Stuart G., Lankester, Arjan C., Boztug, Kaan, İstinye Üniversitesi, Tıp Fakültesi, Dahili Tıp Bilimleri Bölümü, and Erol Cipe, Funda

Subjects
Male, Mononucleosis, medicine.medical_treatment, [SDV]Life Sciences [q-bio], Medizin, CHILDREN, Hematopoietic stem cell transplantation, T-CELL, medicine.disease_cause, Biochemistry, 0302 clinical medicine, immune system diseases, hemic and lymphatic diseases, MEMORY B-CELLS, EPSTEIN-BARR-VIRUS, Child, 0303 health sciences, Hematology, Hematopoietic Stem Cell Transplantation, Allografts, 3. Good health, Survival Rate, 030220 oncology & carcinogenesis, Child, Preschool, Female, Adult, medicine.medical_specialty, Adolescent, Immunology, chemical and pharmacologic phenomena, IMMUNITY, Disease-Free Survival, 03 medical and health sciences, Immune system, EBV, Internal medicine, medicine, Humans, 030304 developmental biology, Retrospective Studies, Hemophagocytic lymphohistiocytosis, business.industry, Genetic Diseases, Inborn, Immunologic Deficiency Syndromes, Infant, STEM-CELL TRANSPLANTATION, Cell Biology, Immune dysregulation, COMBINED IMMUNODEFICIENCY, medicine.disease, REFERENCE VALUES, Lymphoma, Tumor Necrosis Factor Receptor Superfamily, Member 7, business, CD8, GENERATION, CD27 Ligand
Abstract

Biallelic mutations in the genes encoding CD27 or its ligand CD70 underlie inborn errors of immunity (IEIs) characterized predominantly by Epstein-Barr virus (EBV)-associated immune dysregulation, such as chronic viremia, severe infectious mononucleosis, hemophagocytic lymphohistiocytosis (HLH), lymphoproliferation, and malignancy. A comprehensive understanding of the natural history, immune characteristics, and transplant outcomes has remained elusive. Here, in a multi-institutional global collaboration, we collected the clinical information of 49 patients from 29 families (CD27, n = 33; CD70, n = 16), including 24 previously unreported individuals and identified a total of 16 distinct mutations in CD27, and 8 in CD70, respectively. The majority of patients (90%) were EBV+ at diagnosis, but only ∼30% presented with infectious mononucleosis. Lymphoproliferation and lymphoma were the main clinical manifestations (70% and 43%, respectively), and 9 of the CD27-deficient patients developed HLH. Twenty-one patients (43%) developed autoinflammatory features including uveitis, arthritis, and periodic fever. Detailed immunological characterization revealed aberrant generation of memory B and T cells, including a paucity of EBV-specific T cells, and impaired effector function of CD8+ T cells, thereby providing mechanistic insight into cellular defects underpinning the clinical features of disrupted CD27/CD70 signaling. Nineteen patients underwent allogeneic hematopoietic stem cell transplantation (HSCT) prior to adulthood predominantly because of lymphoma, with 95% survival without disease recurrence. Our data highlight the marked predisposition to lymphoma of both CD27- and CD70-deficient patients. The excellent outcome after HSCT supports the timely implementation of this treatment modality particularly in patients presenting with malignant transformation to lymphoma.

Full Text
View/download PDF

Catalog

Books, media, physical & digital resources
See catalog results