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Verfasst von:Böhm, Svea [VerfasserIn]   i
 Mauz-Körholz, Christine [VerfasserIn]   i
 Wustrau, Katharina [VerfasserIn]   i
 Pachlopnik Schmid, Jana [VerfasserIn]   i
 Prader, Seraina [VerfasserIn]   i
 Ahlmann, Martina [VerfasserIn]   i
 Yacobovich, Joanne [VerfasserIn]   i
 Beier, Rita [VerfasserIn]   i
 Speckmann, Carsten [VerfasserIn]   i
 Behnisch, Wolfgang [VerfasserIn]   i
 Ifversen, Marianne [VerfasserIn]   i
 Jordan, Michael [VerfasserIn]   i
 Marsh, Rebecca [VerfasserIn]   i
 Naumann-Bartsch, Nora [VerfasserIn]   i
 Mauz-Körholz, Christine [VerfasserIn]   i
 Hönig, Manfred [VerfasserIn]   i
 Schulz, Ansgar [VerfasserIn]   i
 Malinowska, Iwona [VerfasserIn]   i
 Hines, Melissa [VerfasserIn]   i
 Nichols, Kim E. [VerfasserIn]   i
 Gil-Herrera, Juana [VerfasserIn]   i
 Talano, Julie-An [VerfasserIn]   i
 Crooks, Bruce [VerfasserIn]   i
 Formankova, Renata [VerfasserIn]   i
 Jorch, Norbert [VerfasserIn]   i
 Bakhtiar, Shahrzad [VerfasserIn]   i
 Kühnle, Ingrid [VerfasserIn]   i
 Streiter, Monika [VerfasserIn]   i
 Nathrath, Michaela [VerfasserIn]   i
 Russo, Alexandra [VerfasserIn]   i
 Dürken, Matthias [VerfasserIn]   i
 Lang, Peter [VerfasserIn]   i
 Lindemans, Caroline [VerfasserIn]   i
 Henter, Jan-Inge [VerfasserIn]   i
 Lehmberg, Kai [VerfasserIn]   i
 Ehl, Stephan [VerfasserIn]   i
Titel:Survival in primary hemophagocytic lymphohistiocytosis, 2016 to 2021
Titelzusatz:etoposide is better than its reputation
Verf.angabe:Svea Böhm, Katharina Wustrau, Jana Pachlopnik Schmid, Seraina Prader, Martina Ahlmann, Joanne Yacobovich, Rita Beier, Carsten Speckmann, Wolfgang Behnisch, Marianne Ifversen, Michael Jordan, Rebecca Marsh, Nora Naumann-Bartsch, Christine Mauz-Körholz, Manfred Hönig, Ansgar Schulz, Iwona Malinowska, Melissa Hines, Kim E. Nichols, Juana Gil-Herrera, Julie-An Talano, Bruce Crooks, Renata Formankova, Norbert Jorch, Shahrzad Bakhtiar, Ingrid Kühnle, Monika Streiter, Michaela Nathrath, Alexandra Russo, Matthias Dürken, Peter Lang, Caroline Lindemans, Jan-Inge Henter, Kai Lehmberg, Stephan Ehl
E-Jahr:2024
Jahr:March 7, 2024
Umfang:10 S.
Fussnoten:Gesehen am 13.01.2025
Titel Quelle:Enthalten in: Blood
Ort Quelle:Washington, DC : American Society of Hematology, 1946
Jahr Quelle:2024
Band/Heft Quelle:143(2024), 10, Seite 872-881
ISSN Quelle:1528-0020
Abstract:Primary hemophagocytic lymphohistiocytosis (pHLH) is a life-threatening hyperinflammatory syndrome that develops mainly in patients with genetic disorders of lymphocyte cytotoxicity and X-linked lymphoproliferative syndromes. Previous studies with etoposide-based treatment followed by hematopoetic stem cell transplantation (HSCT) resulted in 5-year survival of 50% to 59%. Contemporary data are lacking. We evaluated 88 patients with pHLH documented in the international HLH registry from 2016-2021. In 12 of 88 patients, diagnosis was made without HLH activity, based on siblings or albinism. Major HLH-directed drugs (etoposide, antithymocyte globulin, alemtuzumab, emapalumab, ruxolitinib) were administered to 66 of 76 patients who were symptomatic (86% first-line etoposide); 16 of 57 patients treated with etoposide and 3 of 9 with other first-line treatment received salvage therapy. HSCT was performed in 75 patients; 7 patients died before HSCT. Three-year probability of survival (pSU) was 82% (confidence interval [CI], 72%-88%) for the entire cohort and 77% (CI, 64%-86%) for patients receiving first-line etoposide. Compared with the HLH-2004 study, both pre-HSCT and post-HSCT survival of patients receiving first-line etoposide improved, 83% to 91% and 70% to 88%. Differences to HLH-2004 included preferential use of reduced-toxicity conditioning and reduced time from diagnosis to HSCT (from 148 to 88 days). Three-year pSU was lower with haploidentical (4 of 9 patients [44%]) than with other donors (62 of 66 [94%]; P < .001). Importantly, early HSCT for patients who were asymptomatic resulted in 100% survival, emphasizing the potential benefit of newborn screening. This contemporary standard-of-care study of patients with pHLH reveals that first-line etoposide-based therapy is better than previously reported, providing a benchmark for novel treatment regimes.
DOI:doi:10.1182/blood.2023022281
URL:Bitte beachten Sie: Dies ist ein Bibliographieeintrag. Ein Volltextzugriff für Mitglieder der Universität besteht hier nur, falls für die entsprechende Zeitschrift/den entsprechenden Sammelband ein Abonnement besteht oder es sich um einen OpenAccess-Titel handelt.

Resolving-System: https://doi.org/10.1182/blood.2023022281
 DOI: https://doi.org/10.1182/blood.2023022281
Datenträger:Online-Ressource
Sprache:eng
K10plus-PPN:1883320968
Verknüpfungen:→ Zeitschrift

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