Search Results - "Lehmberg, Kai"

Hemophagocytic lymphohistiocytosis in adults: collaborative analysis of 137 cases of a nationwide German registry by Birndt, Sebastian, Schenk, Thomas, Heinevetter, Babett, Brunkhorst, Frank M., Maschmeyer, Georg, Rothmann, Frank, Weber, Thomas, Müller, Markus, Panse, Jens, Penack, Olaf, Schroers, Roland, Braess, Jan, Frickhofen, Norbert, Ehl, Stephan, Janka, Gritta, Lehmberg, Kai, Pletz, Mathias W., Hochhaus, Andreas, Ernst, Thomas, La Rosée, Paul

“…Purpose Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome emerging from a deregulated immune response due to various triggers. In…”
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Confirmed efficacy of etoposide and dexamethasone in HLH treatment: long-term results of the cooperative HLH-2004 study by Bergsten, Elisabet, Horne, AnnaCarin, Aricó, Maurizio, Astigarraga, Itziar, Egeler, R. Maarten, Filipovich, Alexandra H., Ishii, Eiichi, Janka, Gritta, Ladisch, Stephan, Lehmberg, Kai, McClain, Kenneth L., Minkov, Milen, Montgomery, Scott, Nanduri, Vasanta, Rosso, Diego, Henter, Jan-Inge

“…Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome comprising familial/genetic HLH (FHL) and secondary HLH. In the…”
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Cell Versus Cytokine - Directed Therapies for Hemophagocytic Lymphohistiocytosis (HLH) in Inborn Errors of Immunity by Wegehaupt, Oliver, Wustrau, Katharina, Lehmberg, Kai, Ehl, Stephan

“…Hemophagocytic lymphohistiocytosis (HLH) is a heterogeneous hyperinflammatory syndrome with different pathways of pathogenesis resulting in similar clinical…”
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Clinical Features, Treatment, and Outcome of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A Multinational, Multicenter Study of 362 Patients by Minoia, Francesca, Davì, Sergio, Horne, AnnaCarin, Demirkaya, Erkan, Bovis, Francesca, Li, Caifeng, Lehmberg, Kai, Weitzman, Sheila, Insalaco, Antonella, Wouters, Carine, Shenoi, Susan, Espada, Graciela, Ozen, Seza, Anton, Jordi, Khubchandani, Raju, Russo, Ricardo, Pal, Priyankar, Kasapcopur, Ozgur, Miettunen, Paivi, Maritsi, Despoina, Merino, Rosa, Shakoory, Bita, Alessio, Maria, Chasnyk, Vyacheslav, Sanner, Helga, Gao, Yi‐Jin, Huasong, Zeng, Kitoh, Toshiyuki, Avcin, Tadej, Fischbach, Michel, Frosch, Michael, Grom, Alexei, Huber, Adam, Jelusic, Marija, Sawhney, Sujata, Uziel, Yosef, Ruperto, Nicolino, Martini, Alberto, Cron, Randy Q., Ravelli, Angelo

“…Objective To describe the clinical, laboratory, and histopathologic features, current treatment, and outcome of patients with macrophage activation syndrome…”
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Consensus recommendations for the diagnosis and management of hemophagocytic lymphohistiocytosis associated with malignancies by Lehmberg, Kai, Nichols, Kim E, Henter, Jan-Inge, Girschikofsky, Michael, Greenwood, Tatiana, Jordan, Michael, Kumar, Ashish, Minkov, Milen, La Rosée, Paul, Weitzman, Sheila

“…The hyperinflammatory syndrome hemophagocytic lymphohistiocytosis can occur in the context of malignancies. Malignancy-triggered hemophagocytic…”
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Differentiating Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis from Other Forms of Hemophagocytic Lymphohistiocytosis by Lehmberg, Kai, MD, Pink, Isabell, Eulenburg, Christine, PhD, Beutel, Karin, MD, Maul-Pavicic, Andrea, PhD, Janka, Gritta, MD

“…Objectives To identify measures distinguishing macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis (sJIA) from familial…”
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Autoinflammatory mutation in NLRC4 reveals a leucine-rich repeat (LRR)–LRR oligomerization interface by Moghaddas, Fiona, Zeng, Ping, Zhang, Yuxia, Schützle, Heike, Brenner, Sebastian, Hofmann, Sigrun R., Berner, Reinhard, Zhao, Yuanbo, Lu, Bingtai, Chen, Xiaoyun, Zhang, Li, Cheng, Suyun, Winkler, Stefan, Lehmberg, Kai, Canna, Scott W., Czabotar, Peter E., Wicks, Ian P., De Nardo, Dominic, Hedrich, Christian M., Zeng, Huasong, Masters, Seth L.

“…Monogenic autoinflammatory disorders are characterized by dysregulation of the innate immune system, for example by gain-of-function mutations in…”
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Inborn errors of immunity with susceptibility to S. aureus infections by Kurz, Hannah, Lehmberg, Kai, Farmand, Susan

“…( ) is a significant human pathogen, in particular in patients with an underlying medical condition. It is equipped with a large variety of virulence factors…”
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A prospective evaluation of degranulation assays in the rapid diagnosis of familial hemophagocytic syndromes by Bryceson, Yenan T., Pende, Daniela, Maul-Pavicic, Andrea, Gilmour, Kimberly C., Ufheil, Heike, Vraetz, Thomas, Chiang, Samuel C., Marcenaro, Stefania, Meazza, Raffaella, Bondzio, Ilka, Walshe, Denise, Janka, Gritta, Lehmberg, Kai, Beutel, Karin, zur Stadt, Udo, Binder, Nadine, Arico, Maurizio, Moretta, Lorenzo, Henter, Jan-Inge, Ehl, Stephan

“…Familial hemophagocytic lymphohistiocytosis (FHL) is a life-threatening disorder of immune regulation caused by defects in lymphocyte cytotoxicity. Rapid…”
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Recommendations for the Use of Etoposide-Based Therapy and Bone Marrow Transplantation for the Treatment of HLH: Consensus Statements by the HLH Steering Committee of the Histiocyte Society by Ehl, Stephan, Astigarraga, Itziar, von Bahr Greenwood, Tatiana, Hines, Melissa, Horne, AnnaCarin, Ishii, Eiichi, Janka, Gritta, Jordan, Michael B., La Rosée, Paul, Lehmberg, Kai, Machowicz, Rafal, Nichols, Kim E., Sieni, Elena, Wang, Zhao, Henter, Jan-Inge

“…Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome requiring aggressive immunosuppressive therapy. Following 2 large…”
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Haematopoietic Stem Cell Transplantation for Primary Haemophagocytic Lymphohistiocytosis by Lehmberg, Kai, Moshous, Despina, Booth, Claire

“…Haematopoietic stem cell transplantation currently remains the only curative treatment of primary forms of haemophagocytic lymphohistiocytosis (HLH). Rapid…”
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The minimum required level of donor chimerism in hereditary hemophagocytic lymphohistiocytosis by Hartz, Bernd, Marsh, Rebecca, Rao, Kanchan, Henter, Jan-Inge, Jordan, Michael, Filipovich, Lisa, Bader, Peter, Beier, Rita, Burkhardt, Birgit, Meisel, Roland, Schulz, Ansgar, Winkler, Beate, Albert, Michael H., Greil, Johann, Karasu, Gülsün, Woessmann, Wilhelm, Corbacioglu, Selim, Gruhn, Bernd, Holter, Wolfgang, Kühl, Jörn-Sven, Lang, Peter, Seidel, Markus G., Veys, Paul, Löfstedt, Alexandra, Ammann, Sandra, Ehl, Stephan, Janka, Gritta, Müller, Ingo, Lehmberg, Kai

“…Reduced-intensity conditioning has improved survival after hematopoietic stem cell transplantation (HSCT) for hemophagocytic lymphohistiocytosis (HLH) at the…”
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Recent advances in the diagnosis and treatment of hemophagocytic lymphohistiocytosis by Bode, Sebastian Fn, Lehmberg, Kai, Maul-Pavicic, Andrea, Vraetz, Thomas, Janka, Gritta, Stadt, Udo Zur, Ehl, Stephan

“…Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening disease of severe hyperinflammation caused by uncontrolled proliferation of activated…”
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Case report: Granulocyte-macrophage colony-stimulating factor sargramostim did not rescue the neutrophil phenotype in two patients with JAGN1-mutant severe congenital neutropenia by Farmand, Susan, Aydin, Susanne Eva, Wustrau, Katharina, Böhm, Svea, Ayuk, Francis, Escherich, Gabriele, Skokowa, Julia, Müller, Ingo, Lehmberg, Kai

“…Homozygous or compound heterozygous mutations in JAGN1 cause severe congenital neutropenia. JAGN1-mutant patients present with severe early-onset bacterial…”
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Mutations in EXTL3 Cause Neuro-immuno-skeletal Dysplasia Syndrome by Oud, Machteld M., Tuijnenburg, Paul, Hempel, Maja, van Vlies, Naomi, Ren, Zemin, Ferdinandusse, Sacha, Jansen, Machiel H., Santer, René, Johannsen, Jessika, Bacchelli, Chiara, Alders, Marielle, Li, Rui, Davies, Rosalind, Dupuis, Lucie, Cale, Catherine M., Wanders, Ronald J.A., Pals, Steven T., Ocaka, Louise, James, Chela, Müller, Ingo, Lehmberg, Kai, Strom, Tim, Engels, Hartmut, Williams, Hywel J., Beales, Phil, Roepman, Ronald, Dias, Patricia, Brunner, Han G., Cobben, Jan-Maarten, Hall, Christine, Hartley, Taila, Le Quesne Stabej, Polona, Mendoza-Londono, Roberto, Davies, E. Graham, de Sousa, Sérgio B., Lessel, Davor, Arts, Heleen H., Kuijpers, Taco W.

“…EXTL3 regulates the biosynthesis of heparan sulfate (HS), important for both skeletal development and hematopoiesis, through the formation of HS proteoglycans…”
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Syntaxin binding mechanism and disease-causing mutations in Munc18-2 by Hackmann, Yvonne, Graham, Stephen C, Ehl, Stephan, Höning, Stefan, Lehmberg, Kai, Aricò, Maurizio, Owen, David J, Griffiths, Gillian M

“…Mutations in either syntaxin 11 (Stx11) or Munc18-2 abolish cytotoxic T lymphocytes (CTL) and natural killer cell (NK) cytotoxicity, and give rise to familial…”
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Allogeneic hematopoietic cell transplantation for XIAP deficiency: an international survey reveals poor outcomes by Marsh, Rebecca A., Rao, Kanchan, Satwani, Prakash, Lehmberg, Kai, Müller, Ingo, Li, Dandan, Kim, Mi-Ok, Fischer, Alain, Latour, Sylvain, Sedlacek, Petr, Barlogis, Vincent, Hamamoto, Kazuko, Kanegane, Hirokazu, Milanovich, Sam, Margolis, David A., Dimmock, David, Casper, James, Douglas, Dorothea N., Amrolia, Persis J., Veys, Paul, Kumar, Ashish R., Jordan, Michael B., Bleesing, Jack J., Filipovich, Alexandra H.

“…There have been no studies on patient outcome after allogeneic hematopoietic cell transplantation (HCT) in patients with X-linked inhibitor of apoptosis (XIAP)…”
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Evolution of disease activity and biomarkers on and off rapamycin in 28 patients with autoimmune lymphoproliferative syndrome by Klemann, Christian, Esquivel, Myrian, Magerus-Chatinet, Aude, Lorenz, Myriam R, Fuchs, Ilka, Neveux, Nathalie, Castelle, Martin, Rohr, Jan, da Cunha, Claudia Bettoni, Ebinger, Martin, Kobbe, Robin, Kremens, Bernhard, Kollert, Florian, Gambineri, Eleonora, Lehmberg, Kai, Seidel, Markus G, Siepermann, Kathrin, Voelker, Thomas, Schuster, Volker, Goldacker, Sigune, Schwarz, Klaus, Speckmann, Carsten, Picard, Capucine, Fischer, Alain, Rieux-Laucat, Frederic, Ehl, Stephan, Rensing-Ehl, Anne, Neven, Benedicte

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Reduced-Intensity/Reduced-Toxicity Conditioning Approaches Are Tolerated in XIAP Deficiency but Patients Fare Poorly with Acute GVHD by Arnold, Danielle E., Nofal, Rofida, Wakefield, Connor, Lehmberg, Kai, Wustrau, Katharina, Albert, Michael H., Morris, Emma C., Heimall, Jennifer R., Bunin, Nancy J., Kumar, Ashish, Jordan, Michael B., Cole, Theresa, Choo, Sharon, Brettig, Tim, Speckmann, Carsten, Ehl, Stephan, Salamonowicz, Malgorzata, Wahlstrom, Justin, Rao, Kanchan, Booth, Claire, Worth, Austen, Marsh, Rebecca A.

“…X-linked inhibitor of apoptosis (XIAP) deficiency is an inherited primary immunodeficiency characterized by chronic inflammasome overactivity and associated…”
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Hemophagocytic Lymphohistiocytosis in Imported Pediatric Visceral Leishmaniasis in a Nonendemic Area by Bode, Sebastian F.N., MD, Bogdan, Christian, MD, Beutel, Karin, MD, Behnisch, Wolfgang, MD, Greiner, Jeanette, MD, Henning, Stephan, MD, Jorch, Norbert, MD, Jankofsky, Martin, MD, Jakob, Marcus, MD, Schmid, Irene, MD, Veelken, Norbert, MD, PhD, Vraetz, Thomas, MD, Janka, Gritta, MD, Ehl, Stephan, MD, Lehmberg, Kai, MD

“…Objectives To describe characteristics of visceral leishmaniasis-associated hemophagocytic lymphohistiocytosis (HLH) with focus on diagnostic clues and…”
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