Search

Your search keyword '"Faraci, Maura"' showing total 28 results
Start Over Author "Faraci, Maura" Journal blood
28 results on '"Faraci, Maura"'

1. Outcomes of HLA-mismatched HSCT with TCRαβ/CD19 depletion or post-HSCT cyclophosphamide for inborn errors of immunity

Author

Lum, Su Han, Albert, Michael H., Gilbert, Patrick, Sirait, Tiarlan, Algeri, Mattia, Muratori, Rafaella, Fournier, Benjamin, Laberko, Alexandra, Karakukcu, Musa, Unal, Elrem, Ayas, Mouhab, Yadav, Satya Prakash, Fisgin, Tunc, Elfeky, Reem, Fernandes, Juliana, Faraci, Maura, Cole, Theresa, Schulz, Ansgar, Meisel, Roland, Zecca, Marco, Ifversen, Marianne, Biffi, Alessandra, Diana, Jean-Sebastien, Vallée, Tanja, Giardino, Stefano, Ersoy, Gizem Zengin, Moshous, Despina, Gennery, Andrew R., Balashov, Dmitry, Bonfim, Carmem, Locatelli, Franco, Lankester, Arjan, Neven, Bénédicte, and Slatter, Mary

Published
2024
Full Text
View/download PDF

3. Outcomes of hematopoietic stem cell transplantation in 813 pediatric patients with Fanconi anemia

Author

Lum, Su Han, Eikema, Dirk-Jan, Piepenbroek, Brian, Wynn, Robert F., Samarasinghe, Sujith, Dalissier, Arnaud, Kalwak, Krysztof, Ayas, Mouhab, Hamladji, Rose-Marie, Yesilipek, Akif, Dalle, Jean-Hugues, Uckan-Cetinkaya, Duygu, Bierings, Marc, Kupesiz, Alphan, Halahleh, Khalid, Skorobogatova, Elena, Öztürk, Gülyüz, Faraci, Maura, Renard, Cecile, Evans, Pamela, Corbacioglu, Selim, Locatelli, Franco, Dufour, Carlo, Risitano, Antonio, and Peffault de Latour, Régis

Published
2024
Full Text
View/download PDF

4. Hematopoietic stem cell transplantation for adolescents and adults with inborn errors of immunity: an EBMT IEWP study

Author

Aiuti, Alessandro, Maschan, Alexei, Aljurf, Mahmoud, Gedde-Dahl, Tobias, Gurman, Gunhan, Bordon, Victoria, Kriván, Gergely, Locatelli, Franco, Porta, Fulvio, Valcárcel, David, Beguin, Yves, Faraci, Maura, Kröger, Nicolaus, Kulagin, Aleksandr, Shaw, Peter J., Veelken, Joan Hendrik, Diaz de Heredia, Cristina, Fagioli, Franca, Felber, Matthias, Gruhn, Bernd, Holter, Wolfgang, Rössig, Claudia, Sedlacek, Petr, Apperley, Jane, Ayas, Mouhab, Bodova, Ivana, Choi, Goda, Cornelissen, J.J., Sirvent, Anne, Khan, Anjum, Kupesiz, Alphan, Lenhoff, Stig, Ozdogu, Hakan, von der Weid, Nicolas, Rovira, Montserrat, Schots, Rik, Vinh, Donald C., Albert, Michael H., Sirait, Tiarlan, Eikema, Dirk-Jan, Bakunina, Katerina, Wehr, Claudia, Suarez, Felipe, Fox, Maria Laura, Mahlaoui, Nizar, Gennery, Andrew R., Lankester, Arjan C., Beier, Rita, Bernardo, Maria Ester, Bigley, Venetia, Lindemans, Caroline A., Burns, Siobhan O., Carpenter, Ben, Dybko, Jaroslaw, Güngör, Tayfun, Hauck, Fabian, Lum, Su Han, Balashov, Dmitry, Meisel, Roland, Moshous, Despina, Schulz, Ansgar, Speckmann, Carsten, Slatter, Mary A., Strahm, Brigitte, Uckan-Cetinkaya, Duygu, Meyts, Isabelle, Vallée, Tanja C., Wynn, Robert, Neven, Bénédicte, and Morris, Emma C.

Published
2022
Full Text
View/download PDF

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

Author

Ghosh, Sujal, Köstel Bal, Sevgi, Edwards, Emily S.J., Pillay, Bethany, Jiménez Heredia, Raúl, Erol Cipe, Funda, 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, Krüger, 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, Dückers, Gregor, Becker, Ruy Perez, von Bernuth, Horst, Latour, Sylvain, Faraci, Maura, Gattorno, Marco, Su, Helen C., Pan-Hammarström, Qiang, Hammarström, Lennart, Lenardo, Michael J., Ma, Cindy S., Niehues, Tim, Aghamohammadi, Asghar, Rezaei, Nima, Ikinciogullari, Aydan, Tangye, Stuart G., Lankester, Arjan C., and Boztug, Kaan

Published
2020
Full Text
View/download PDF

6. Interleukin-1 blockade in patients with Wiskott-Aldrich syndrome: a retrospective multinational case series

Author

Naviglio, Samuele, Cicalese, Maria Pia, Rivers, Elizabeth, Ferrua, Francesca, Bonfim, Carmem, Cenciarelli, Sabina, Cheong, Kai-Ning, Faraci, Maura, Giardino, Stefano, Ghosh, Sujal, Lee, Pamela P., Lyra, Paula Teixeira, Meisel, Roland, Sofia, Valentina, Tessitore, Antimo, Tommasini, Alberto, Valencic, Erica, Vallée, Tanja Christine, Volpi, Stefano, Worth, Austen J., Rabusin, Marco, Albert, Michael H., Thrasher, Adrian J., and Aiuti, Alessandro

Published
2024
Full Text
View/download PDF

7. HLA-Haploidentical Stem Cell Transplantation in Children with Inherited Bone Marrow Failure Syndromes: A Retrospective Analysis on Behalf of EBMT Severe Aplastic Anemia Working Party

Author

Giardino, Stefano, primary, Eikema, Dirk-Jan, additional, Piepenbroek, Brian, additional, Algeri, Mattia, additional, Ayas, Mouhab, additional, Faraci, Maura, additional, Tbakhi, Abdelghani, additional, Zecca, Marco, additional, Essa, Mohammed, additional, Neven, Bénédicte, additional, Bertrand, Yves, additional, Kharya, Gaurav, additional, Bykova, Tatiana A, additional, Lawson, Sarah, additional, Petrini, Mario, additional, Mohseny, Alexander, additional, Rialland, Fanny, additional, James, Beki, additional, Colita, Anca, additional, Fahd, Mony, additional, Cesaro, Simone, additional, Schulz, Ansgar, additional, Dufour, Carlo, additional, Risitano, Antonio, additional, and Peffault de Latour, Régis, additional

Published
2022
Full Text
View/download PDF

8. Ruxolitinib in Pediatric Patients with Treatment-Naïve or Steroid-Refractory Acute Graft-Versus-Host Disease: Primary Findings from the Phase I/II REACH4 Study

Author

Locatelli, Franco, primary, Kang, Hyoung Jin, additional, Bruno, Bénédicte, additional, Gandemer, Virginie, additional, Rialland, Fanny, additional, Faraci, Maura, additional, Takahashi, Yoshiyuki, additional, Koh, Katsuyoshi, additional, Bittencourt, Henrique, additional, Cleary, Grace, additional, Rosko, Christine, additional, Roussou, Pantelia, additional, St. Pierre, Annie, additional, Prahallad, Anirudh, additional, and Díaz-de-Heredia, Cristina, additional

Published
2022
Full Text
View/download PDF

9. Specific Cytogenetic Abnormalities at Diagnosis Predict Survival after Hematopoietic Cell Transplant in Poor-Risk Pediatric Acute Myeloid Leukemia: A PDWP/EBMT Study

Author

Sharma, Akshay, primary, Galimard, Jacques-Emmanuel, additional, Pryce, Angharad, additional, Bhoopalan, Senthil, additional, Dalissier, Arnaud, additional, Dalle, Jean-Hugues, additional, Locatelli, Franco, additional, Jubert, Charlotte, additional, Mirci-Danicar, Oana, additional, Kitra-Roussou, Vassiliki, additional, Bertrand, Yves, additional, Fagioli, Franca, additional, Rialland, Fanny, additional, Biffi, Alessandra, additional, Wynn, Robert F, additional, Michel, Gerard, additional, Tambaro, Francesco Paolo P, additional, Ahmari, Ali Abdallah, additional, Tbakhi, Abdelghani, additional, Furness, Caroline, additional, Díaz Pérez, Miguel Ángel, additional, Sedlacek, Petr, additional, Bodova, Ivana, additional, Faraci, Maura, additional, Rao, Kanchan, additional, Rubio, Marie Thérèse, additional, Gibson, Brenda, additional, Bhatt, Neel S., additional, and Corbacioglu, Selim, additional

Published
2022
Full Text
View/download PDF

10. Outcome of Haematopoietic Cell Transplantation in 813 Children with Fanconi Anaemia: A Study on Behalf of the EBMT Severe Aplastic Anaemia Working Party and Paediatric Disease Working Party

Author

Lum, Su Han, primary, Samarasinghe, Sujith, additional, Eikema, Dirk-Jan, additional, Piepenbroek, Brian, additional, Dalissier, Arnaud, additional, Ayas, Mouhab, additional, Mousavi, Ashrafsadat, additional, Hamladji, Rose-Marie, additional, Yesilipek, Akif, additional, Dalle, Jean-Hugues, additional, Kansoy, Savas, additional, Locatelli, Franco, additional, Çetinkaya, Duygu, additional, Bierings, Marc, additional, Kupesiz, O. Alphan, additional, Tbakhi, Abdelghani, additional, Skorobogatova, Elena, additional, Öztürk, Gülyüz, additional, Faraci, Maura, additional, Bertrand, Yves, additional, Evans, Pamela, additional, Carbacioglu, Selim, additional, Dufour, Carlo, additional, Risitano, Antonio, additional, Wynn, Robert F, additional, and Peffault de Latour, Régis, additional

Published
2022
Full Text
View/download PDF

11. Outcomes of Allogeneic Hematopoietic Stem Cell Transplantation for Congenital Amegakaryocytic Thrombocytopenia, a PDWP/EBMT Study

Author

Aldebert, Clemence, primary, Fahd, Mony, additional, Galimard, Jacques-Emmanuel, additional, Ghemlas, Ibrahim A., additional, Zecca, Marco, additional, Silva, Juliana, additional, Mohseny, Alexander, additional, Kupesiz, Alphan, additional, Hamladji, Rose-Marie, additional, Miranda, Nuno, additional, Gungor, Tayfun, additional, Wynn, Robert F, additional, Merli, Pietro, additional, Sundin, Mikael, additional, Faraci, Maura, additional, Díaz-de-Heredia, Cristina, additional, Burkhardt, Birgit, additional, Bordon, Victoria, additional, Jubert, Charlotte, additional, Bader, Peter, additional, Ifversen, Marianne, additional, Herrera Arroyo, Concepcion, additional, Maximova, Natalia, additional, Riesco, Susana, additional, Stein, Jerry, additional, Dalissier, Arnaud, additional, Locatelli, Franco, additional, Kalwak, Krzysztof, additional, Dalle, Jean-Hugues, additional, and Corbacioglu, Selim, additional

Published
2022
Full Text
View/download PDF

12. Hematopoietic stem cell transplantation for adolescents and adults with inborn errors of immunity: an EBMT IEWP study

Author

Albert, Michael H., primary, Sirait, Tiarlan, additional, Eikema, Dirk-Jan, additional, Bakunina, Katerina, additional, Wehr, Claudia, additional, Suarez, Felipe, additional, Fox, Maria Laura, additional, Mahlaoui, Nizar, additional, Gennery, Andrew R., additional, Lankester, Arjan C., additional, Beier, Rita, additional, Bernardo, Maria Ester, additional, Bigley, Venetia, additional, Lindemans, Caroline A., additional, Burns, Siobhan O., additional, Carpenter, Ben, additional, Dybko, Jaroslaw, additional, Güngör, Tayfun, additional, Hauck, Fabian, additional, Lum, Su Han, additional, Balashov, Dmitry, additional, Meisel, Roland, additional, Moshous, Despina, additional, Schulz, Ansgar, additional, Speckmann, Carsten, additional, Slatter, Mary A., additional, Strahm, Brigitte, additional, Uckan-Cetinkaya, Duygu, additional, Meyts, Isabelle, additional, Vallée, Tanja C., additional, Wynn, Robert, additional, Neven, Bénédicte, additional, Morris, Emma C., additional, Aiuti, Alessandro, additional, Maschan, Alexei, additional, Aljurf, Mahmoud, additional, Gedde-Dahl, Tobias, additional, Gurman, Gunhan, additional, Bordon, Victoria, additional, Kriván, Gergely, additional, Locatelli, Franco, additional, Porta, Fulvio, additional, Valcárcel, David, additional, Beguin, Yves, additional, Faraci, Maura, additional, Kröger, Nicolaus, additional, Kulagin, Aleksandr, additional, Shaw, Peter J., additional, Veelken, Joan Hendrik, additional, Diaz de Heredia, Cristina, additional, Fagioli, Franca, additional, Felber, Matthias, additional, Gruhn, Bernd, additional, Holter, Wolfgang, additional, Rössig, Claudia, additional, Sedlacek, Petr, additional, Apperley, Jane, additional, Ayas, Mouhab, additional, Bodova, Ivana, additional, Choi, Goda, additional, Cornelissen, J.J., additional, Sirvent, Anne, additional, Khan, Anjum, additional, Kupesiz, Alphan, additional, Lenhoff, Stig, additional, Ozdogu, Hakan, additional, von der Weid, Nicolas, additional, Rovira, Montserrat, additional, Schots, Rik, additional, and Vinh, Donald C., additional

Published
2022
Full Text
View/download PDF

13. Outcome of Allogeneic HSCT after Chemo-Based Conditioning in Infants with Acute Myeloid Leukemia in First Complete Remission: A Multicenter EBMT-PDWP Study

Author

Willasch, Andre Manfred, primary, Peters, Christina, additional, Balduzzi, Adriana, additional, Dalle, Jean-Hugues, additional, Zecca, Marco, additional, Al-Seraihy, Amal, additional, Locatelli, Franco, additional, Bertrand, Yves, additional, Robinson, Stephen, additional, OBrien, Tracey, additional, Toren, Amos, additional, Vujic, Dragana S., additional, Bay, Jacques-Olivier, additional, Jubert, Charlotte, additional, Faraci, Maura, additional, Rialland, Fanny, additional, Kitra-Roussou, Vassiliki, additional, Michel, Gerard, additional, Menconi, Mariacristina, additional, Fagioli, Franca, additional, Ziino, Ottavio, additional, Sundin, Mikael, additional, Pochon, Cecile, additional, Veys, Paul, additional, Moraleda, Jose Maria, additional, Biffi, Alessandra, additional, Dalissier, Arnaud, additional, Corbacioglu, Selim, additional, Ngoya, Maud, additional, Galimard, Jacques-Emmanuel, additional, and Bader, Peter, additional

Published
2021
Full Text
View/download PDF

14. Treatment Duration, Symptom Resolution, and Survival in Defibrotide-Treated Patients with Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome after Hematopoietic Cell Transplantation: Analysis of a Multinational, Prospective, Observational Registry Study

Author

Locatelli, Franco, primary, Faraci, Maura, additional, Yakoub-Agha, Ibrahim, additional, Girault, Stephane, additional, Wheeler, Kate, additional, Ryan, Robert J., additional, Hanvesakul, Raj, additional, and Mohty, Mohamad, additional

Published
2020
Full Text
View/download PDF

15. Ruxolitinib in Pediatric Patients with Treatment-Naïveor Steroid-Refractory Acute Graft-Versus-Host Disease: Primary Findings from the Phase I/II REACH4 Study

Author

Locatelli, Franco, Kang, Hyoung Jin, Bruno, Bénédicte, Gandemer, Virginie, Rialland, Fanny, Faraci, Maura, Takahashi, Yoshiyuki, Koh, Katsuyoshi, Bittencourt, Henrique, Cleary, Grace, Rosko, Christine, Roussou, Pantelia, St. Pierre, Annie, Prahallad, Anirudh, and Díaz-de-Heredia, Cristina

Published
2022
Full Text
View/download PDF

16. Outcome of Transformed Fanconi Anaemia Patients after Hematopoietic Stem Cell Transplantation: Analysis on Behalf of European Group for Blood and Marrow Transplantation

Author

Giardino, Stefano, primary, Eikema, Dirk-Jan, additional, Peffault De Latour, Regis, additional, Bertrand, Yves, additional, Aljurf, Mahmoud, additional, Tbakhi, Abdelghani, additional, Holter, Wolfgang, additional, Bornhäsuer, Martin, additional, Rössig, Claudia, additional, Zecca, Marco, additional, Michel, Gerard, additional, Ganser, Arnold, additional, Afanasiev, Boris, additional, Al-Seraihy, Amal, additional, Bosman, Paul, additional, Miano, Maurizio, additional, Pierri, Filomena, additional, Faraci, Maura, additional, Lanino, Edoardo, additional, Risitano, Antonio M., additional, Kröger, Nicolaus, additional, and Dufour, Carlo, additional

Published
2018
Full Text
View/download PDF

19. Single-Lineage Bone Marrow Failure Driven By 2 Novel PI3KCD Mutations

Author

Miano, Maurizio, primary, Fioredda, Francesca, additional, Grossi, Alice, additional, Cappelli, Enrico, additional, Palmisani, Elena, additional, Faraci, Maura, additional, Lanza, Tiziana, additional, Terranova, Paola, additional, Calvillo, Michaela, additional, Micalizzi, Concetta, additional, Lanino, Edoardo, additional, Pierri, Filomena, additional, Giardino, Stefano, additional, Ceccherini, Isabella, additional, and Dufour, Carlo, additional

Published
2016
Full Text
View/download PDF

20. Defibrotide for the Treatment of Hepatic Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome Following Hematopoietic Stem Cell Transplantation or Chemotherapy--Results from the Italian Therapeutic Use Protocol

Author

Locatelli, Franco, primary, Faraci, Maura, additional, Cesaro, Simone, additional, Pagliara, Daria, additional, Fagioli, Franca, additional, Zecca, Marco, additional, Amber, Vian, additional, Banerjee, Kamalika, additional, Finetto, Giorgia, additional, and Ciceri, Fabio, additional

Published
2015
Full Text
View/download PDF

21. Evaluation of Clinical and Morphological Practices in the Diagnosis of Transplant-Associated Microangiopathy: A Study on Behalf of the Complications and Quality of Life Working Party of the EBMT

Author

Moiseev, Ivan S, Tsvetkova, Tatyana, Aljurf, Mahmoud, Alnounou, Randa M, Bogardt, Janet, Chalandon, Yves, Drokov, Mikhail Yu., Dvirnyk, Valentina, Faraci, Maura, Friis, Lone Smidstrup, Giglio, Fabio, Greinix, Hildegard T., Kornblit, Brian Thomas, Külper, Christiane, Lewandowski, Krzysztof, Neumeister, Peter, Niederwieser, Dietger, Passweg, Jakob R., Penack, Olaf, Peric, Zinaida, Piekarska, Agnieszka, Ronchi, Paola Erminia, Rovó, Alicia, Rzepecki, Piotr, Scuderi, Francesca, Sigrist, Daniel, Siitonen, Sanna M., Stoelzel, Friedrich, Sulek, Kazimierz, Tsakiris, Dimitrios A., Wilkowojska, Urszula, Duarte, Rafael F, Ruutu, Tapani, and Basak, Grzegorz W

Published
2017
Full Text
View/download PDF

22. Impact Of Prophylaxis With Defibrotide On The Occurrence Of Acute GvHD In Allogeneic HSCT

Author

Corbacioglu, Selim, primary, Cesaro, Simone, additional, Faraci, Maura, additional, Gruhn, Bernd, additional, Boelens, Jaap J, additional, Schulz, Ansgar, additional, Mueller, Ingo, additional, Stein, Jerry, additional, Wynn, Robert, additional, Greil, Johann, additional, Sykora, Karl-Walter, additional, Toporski, Jacek, additional, Sedlacek, Petr, additional, Schlegel, Paul G., additional, Ehlert, Karoline, additional, Mauz-Koerholz, Christine, additional, Bader, Peter, additional, Tudone, Elena, additional, Ballabio, Maria, additional, Heringa, Carin, additional, and Peters, Christina, additional

Published
2013
Full Text
View/download PDF

23. New autoimmune diseases after cord blood transplantation: a retrospective study of EUROCORD and the Autoimmune Disease Working Party of the European Group for Blood and Marrow Transplantation

Author

Daikeler, Thomas, primary, Labopin, Myriam, additional, Ruggeri, Annalisa, additional, Crotta, Alessandro, additional, Abinun, Mario, additional, Hussein, Ayad Ahmed, additional, Carlson, Kristina, additional, Cornillon, Jérôme, additional, Diez-Martin, Jose L., additional, Gandemer, Virginie, additional, Faraci, Maura, additional, Lindemans, Caroline, additional, O'Meara, Anne, additional, Mialou, Valerie, additional, Renard, Marleen, additional, Sedlacek, Petr, additional, Sirvent, Anne, additional, Socié, Gérard, additional, Sora, Federica, additional, Varotto, Stefania, additional, Sanz, Jaime, additional, Voswinkel, Jan, additional, Vora, Ajay, additional, Yesilipek, M. Akif, additional, Herr, Andree-Laure, additional, Gluckman, Eliane, additional, Farge, Dominique, additional, and Rocha, Vanderson, additional

Published
2013
Full Text
View/download PDF

24. Incidence and Risk Factors for Secondary Autoimmune Diseases (AD) After Cord Blood Transplantation (CBT). Retrospective Analysis on Behalf of Eurocord and the EBMT Autoimmune Diseases Working Party.

Author

Daikeler, Thomas, primary, Labopin, Myriam, additional, Socié, Gérard, additional, Faraci, Maura, additional, Abdel-Rahman, Fawzi, additional, Yesilipek, M. Akif, additional, Arcese, William, additional, O'Meara, Anne, additional, Sirvent, Anne, additional, Voswinkel, Jan, additional, Verdeguer, Amparo, additional, Gandemer, Virginie, additional, Schoemans, Hélène, additional, Cant, Andrew J., additional, Messina, Chiara, additional, Sedlacek, Petr, additional, Vora, Ajay J., additional, Mialou, Valerie, additional, Díez, Jose Luis, additional, Sorà, Federica, additional, Jubert, Charlotte, additional, Herr, Andrée-Laure, additional, Ruggeri, Annalisa, additional, Crotta, Alessandro, additional, Gluckman, Eliane, additional, Farge, Dominique, additional, and Rocha, Vanderson, additional

Published
2010
Full Text
View/download PDF

25. Defibrotide (DF) for the Prevention of Hepatic Veno-Occlusive Disease (VOD) in Pediatric Stem Cell Transplantion: Results of a Prospective Phase II/III Randomized, Multicenter Study.

Author

Corbacioglu, Selim, primary, Cesaro, Simone, additional, Faraci, Maura, additional, Valteau-Couanet, Dominique, additional, Gruhn, Bernd, additional, Boelens, Jaap Jan, additional, Uderzo, Cornelio, additional, Hewitt, Netty, additional, Schrum, Johanna, additional, Mueller, Ingo, additional, Schulz, Ansgar S, additional, Stein, Jerry, additional, Wynn, Robert, additional, Greil, Johann, additional, Matthes, Susanne, additional, Karl-Walter, Sykora, additional, Fuehrer, Monika, additional, O'Meare, Anne, additional, Debatin, Klaus-Michael, additional, Massaro, Joseph, additional, D'Agostino, Ralph, additional, Hoyle, Margeret, additional, Iacobelli, Massimo, additional, Peters, Christina, additional, and Dini, Giorgio, additional

Published
2009
Full Text
View/download PDF

26. Incidence of Veno-Occlusive Disease with IV in Busulfan Children Is Higher Than Expected: Preliminary Results of the VOD-DF Trial.

Author

Corbacioglu, Selim, primary, Cesaro, Simone, additional, Faraci, Maura, additional, Gruhn, Bernd, additional, Boelens, Jaap Jan, additional, Uderzo, Cornelio, additional, Hewitt, Netty, additional, Schrum, Johanna, additional, Mueller, Ingo, additional, Schulz, Ansgar S, additional, Stein, Jerry, additional, Wynn, Robert, additional, Greil, Johann, additional, Matthes, Susanne, additional, Sykora, Karl-Walter, additional, Fuehrer, Monika, additional, Toporski, Jacek, additional, Sedlacek, Petr, additional, Muche, Rainer, additional, Rohlmann, Friederike, additional, and Peters, Christina, additional

Published
2009
Full Text
View/download PDF

27. Cardiac and Pulmonary Function Late Effects after Bone Marrow Transplantation in Childhood: Prospective Study on Behalf of EBMT LEWP Group.

Author

Uderzo, Cornelio, primary, Corti, Paola, primary, Fedeli, Sara, primary, Tana, Francesco, primary, Messina, Chiara, primary, Pillon, Marta, primary, Tridello, Gloria, primary, Galambrun, Claire, primary, Zintl, Felix, primary, Dini, Giorgio, primary, Faraci, Maura, primary, Fagioli, Franca, primary, Nysom, Karsten, primary, Verdeguer, Amparo, primary, Urban, Christian, primary, Tichelli, André, primary, and Socié, Gerard, primary

Published
2005
Full Text
View/download PDF

28. 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