Search Results - "Stepensky, P."

The syndrome of hemophagocytic lymphohistiocytosis in primary immunodeficiencies: implications for differential diagnosis and pathogenesis by Bode, Sebastian Fn, Ammann, Sandra, Al-Herz, Waleed, Bataneant, Mihaela, Dvorak, Christopher C, Gehring, Stephan, Gennery, Andrew, Gilmour, Kimberly C, Gonzalez-Granado, Luis I, Groß-Wieltsch, Ute, Ifversen, Marianne, Lingman-Framme, Jenny, Matthes-Martin, Susanne, Mesters, Rolf, Meyts, Isabelle, van Montfrans, Joris M, Pachlopnik Schmid, Jana, Pai, Sung-Yun, Soler-Palacin, Pere, Schuermann, Uta, Schuster, Volker, Seidel, Markus G, Speckmann, Carsten, Stepensky, Polina, Sykora, Karl-Walter, Tesi, Bianca, Vraetz, Thomas, Waruiru, Catherine, Bryceson, Yenan T, Moshous, Despina, Lehmberg, Kai, Jordan, Michael B, Ehl, Stephan

“…Hemophagocytic lymphohistiocytosis is a hyperinflammatory syndrome defined by clinical and laboratory criteria. Current criteria were created to identify…”
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Early lymphocyte collection for anti‐CD19 CART production improves T‐cell fitness in patients with relapsed/refractory diffuse large B‐cell lymphoma by Dubnikov Sharon, T., Assayag, M., Avni, B., Kfir‐Erenfeld, S., Lebel, E., Gatt, M. E., Goldschmidt, N., Stepensky, P., Asherie, N., Grisariu, S.

“…Summary Background Chimeric antigen receptor (CAR) T cells targeted to the CD19 B‐cell antigen form an approved treatment for patients with relapsed/refractory…”
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Incidence and risk factors for moderate-to-severe veno-occlusive disease of the liver after allogeneic stem cell transplantation using a reduced intensity conditioning regimen by Tsirigotis, P D, Resnick, I B, Avni, B, Grisariu, S, Stepensky, P, Or, R, Shapira, M Y

“…The incidence and outcome of moderate-to-severe veno-occlusive (VOD) disease was analyzed in 271 consecutive patients with hematological malignancies who…”
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Inherited susceptibility to pre B-ALL caused by germline transmission of PAX5 c.547G>A by Auer, F, Rüschendorf, F, Gombert, M, Husemann, P, Ginzel, S, Izraeli, S, Harit, M, Weintraub, M, Weinstein, O Y, Lerer, I, Stepensky, P, Borkhardt, A, Hauer, J

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The use of whole exome sequencing for the diagnosis of autosomal recessive malignant infantile osteopetrosis by Shamriz, O., Shaag, A., Yaacov, B., NaserEddin, A., Weintraub, M., Elpeleg, O., Stepensky, P.

“…Autosomal recessive malignant infantile osteopetrosis is a congenital disease characterized by pathologically increased bone density. Recently, the use of…”
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Management of XLP-1 and ITK deficiency: The challenges posed by PID with an unpredictable spectrum of disease manifestations by Shadur, B., Abuzaitoun, O., NaserEddin, A., Even-Or, E., Zaidman, I., Stepensky, P.

“…The incorporation of next generation sequencing into routine immunological practice has enabled the identification of novel inborn errors of disease, helped…”
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The molecular rationale for steroid based therapy of leukemia: diagnostic and therapeutic implications by Yefenof, E, Stepensky, P, Shlomit, K

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Loss-of-function mutations within the IL-2 inducible kinase ITK in patients with EBV-associated lymphoproliferative diseases by Linka, R M, Risse, S L, Bienemann, K, Werner, M, Linka, Y, Krux, F, Synaeve, C, Deenen, R, Ginzel, S, Dvorsky, R, Gombert, M, Halenius, A, Hartig, R, Helminen, M, Fischer, A, Stepensky, P, Vettenranta, K, Köhrer, K, Ahmadian, M R, Laws, H-J, Fleckenstein, B, Jumaa, H, Latour, S, Schraven, B, Borkhardt, A

“…The purpose of this study was the appraisal of the clinical and functional consequences of germline mutations within the gene for the IL-2 inducible T-cell…”
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Non-fermentative Gram-negative rods bacteremia in children with cancer: a 14-year single-center experience by Averbuch, D., Avaky, C., Harit, M., Stepensky, P., Fried, I., Ben-Ami, T., Temper, V., Peled, Y., Troen, H., Masarwa, R., Abu Ahmad, W., Weintraub, M., Revel-Vilk, S., Engelhard, D.

“…Purpose Data on non-fermentative Gram-negative rods (NFGNR) bacteremia in children with malignancies are limited. The aim of this study was to present clinical…”
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Extending the clinical and immunological phenotype of human interleukin-21 receptor deficiency by Stepensky, Polina, Keller, Baerbel, Abuzaitoun, Omar, Shaag, Avraham, Yaacov, Barak, Unger, Susanne, Seidl, Maximilian, Rizzi, Marta, Weintraub, Michael, Elpeleg, Orly, Warnatz, Klaus

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P887: IN‐HOUSE ANTI BCMA CAR‐T THERAPY FOR THE TREATMENT OF RELAPSED REFRACTORY MULTIPLE MYELOMA: PHASE 1 RESULTS by Asherie, N., Kfir‐Erenfeld, S., Avni, B., Grisariu, S., Assayag, M., Sharon, T., Lebel, E., Cohen, C. J., Gatt, M. E., Stepensky, P.

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Deregulation of Fas ligand expression as a novel cause of autoimmune lymphoproliferative syndrome-like disease by Nabhani, Schafiq, Ginzel, Sebastian, Miskin, Hagit, Revel-Vilk, Shoshana, Harlev, Dan, Fleckenstein, Bernhard, Hönscheid, Andrea, Oommen, Prasad T, Kuhlen, Michaela, Thiele, Ralf, Laws, Hans-Jürgen, Borkhardt, Arndt, Stepensky, Polina, Fischer, Ute

“…Autoimmune lymphoproliferative syndrome is frequently caused by mutations in genes involved in the Fas death receptor pathway, but for 20-30% of patients the…”
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Colistin is relatively safe in hematological malignancies and hematopoietic stem cell transplantation patients by Averbuch, D., Horwitz, E., Strahilevitz, J., Stepensky, P., Goldschmidt, N., Gatt, M. E., Shapira, M. Y., Resnick, I. B., Engelhard, D.

“…Purpose Colistin is increasingly used as the last-resort treatment option against infections caused by multidrug-resistant (MDR) Gram-negative pathogens, but…”
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Successful second haploidentical SCT in osteopetrosis by Stepensky, P, Schulz, A S, Lahr, G, Simanovsky, N, Brooks, R, Samuel, S, Or, R, Weintraub, M, Resnick, I

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MSC for the improvement of hematopoietic engraftment by Resnick, I, Stepensky, P, Elkin, G, Barkatz, C, Gurevich, O, Prigozhina, T, Pikarsky, E, Waldman, E, Amar, A, Samuel, S, Shapira, M, Weintraub, M, Or, R

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Combination of umbilical cord blood with BM from a 2-month-old sibling as lifesaving BMT for very severe aplastic anemia by Stepensky, P, Revel-Vilk, S, Weintraub, M, Waldman, E, Harit, M, Averbuch, D, Weinberger, J, Or, R, Resnick, I

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788 - The molecular rationale for steroid based therapy of leukemia: diagnostic and therapeutic implications by Yefenof, E., Stepensky, P., Shlomit, K.

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Long-term outcome of LRBA deficiency in 76 patients after various treatment modalities as evaluated by the immune deficiency and dysregulation activity (IDDA) score by Tesch, Victoria Katharina, Abolhassani, Hassan, Shadur, Bella, Zobel, Joachim, Mareika, Yuliya, Sharapova, Svetlana, Karakoc-Aydiner, Elif, Rivière, Jacques G., Garcia-Prat, Marina, Moes, Nicolette, Haerynck, Filomeen, Gonzales-Granado, Luis I., Santos Pérez, Juan Luis, Mukhina, Anna, Shcherbina, Anna, Aghamohammadi, Asghar, Hammarström, Lennart, Dogu, Figen, Haskologlu, Sule, İkincioğulları, Aydan İ., Köstel Bal, Sevgi, Baris, Safa, Kilic, Sara Sebnem, Karaca, Neslihan Edeer, Kutukculer, Necil, Girschick, Hermann, Kolios, Antonios, Keles, Sevgi, Uygun, Vedat, Stepensky, Polina, Worth, Austen, van Montfrans, Joris M., Peters, Anke M.J., Meyts, Isabelle, Adeli, Mehdi, Marzollo, Antonio, Padem, Nurcicek, Khojah, Amer M., Chavoshzadeh, Zahra, Avbelj Stefanija, Magdalena, Bakhtiar, Shahrzad, Florkin, Benoit, Meeths, Marie, Gamez, Laura, Grimbacher, Bodo, Seppänen, Mikko R.J., Lankester, Arjan, Gennery, Andrew R., Seidel, Markus G.

“…Recent findings strongly support hematopoietic stem cell transplantation (HSCT) in patients with severe presentation of LPS-responsive beige-like anchor…”
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Hematopoietic cell transplantation in chronic granulomatous disease: a study of 712 children and adults by Chiesa, Robert, Wang, Junfeng, Blok, Henric-Jan, Hazelaar, Sheree, Neven, Benedicte, Moshous, Despina, Schulz, Ansgar, Hoenig, Manfred, Hauck, Fabian, Al Seraihy, Amal, Gozdzik, Jolanta, Ljungman, Per, Lindemans, Caroline A., Fernandes, Juliana F., Kalwak, Krzysztof, Strahm, Brigitte, Schanz, Urs, Sedlacek, Petr, Sykora, Karl-Walter, Aksoylar, Serap, Locatelli, Franco, Stepensky, Polina, Wynn, Robert, Lum, Su Han, Zecca, Marco, Porta, Fulvio, Taskinen, Mervi, Gibson, Brenda, Matthes, Susanne, Karakukcu, Musa, Hauri-Hohl, Mathias, Veys, Paul, Gennery, Andrew R., Lucchini, Giovanna, Felber, Matthias, Albert, Michael H., Balashov, Dmitry, Lankester, Arjan, Güngör, Tayfun, Slatter, Mary A.

“…Chronic granulomatous disease (CGD) is a primary immunodeficiency resulting in life-threatening infections and inflammatory complications. Allogeneic…”
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Reduced-intensity conditioning and HLA-matched haemopoietic stem-cell transplantation in patients with chronic granulomatous disease: a prospective multicentre study by Güngör, Tayfun, Dr, Teira, Pierre, MD, Slatter, Mary, MBChB, Stussi, Georg, MD, Stepensky, Polina, MD, Moshous, Despina, MD, Vermont, Clementien, MD, Ahmad, Imran, MD, Shaw, Peter J, FRACP, da Cunha, José Marcos Telles, MD, Schlegel, Paul G, MD, Hough, Rachel, MD, Fasth, Anders, Prof, Kentouche, Karim, MD, Gruhn, Bernd, MD, Fernandes, Juliana F, MD, Lachance, Silvy, FRCP, Bredius, Robbert, MD, Resnick, Igor B, MD, Belohradsky, Bernd H, Prof, Gennery, Andrew, MD, Fischer, Alain, Prof, Gaspar, H Bobby, Prof, Schanz, Urs, MD, Seger, Reinhard, MD, Rentsch, Katharina, PhD, Veys, Paul, FRCP, Haddad, Elie, MD, Albert, Michael H, MD, Hassan, Moustapha, Prof

“…Summary Background In chronic granulomatous disease allogeneic haemopoietic stem-cell transplantation (HSCT) in adolescents and young adults and patients with…”
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