Academic Journal
Allogeneic hematopoietic cell transplantation in advanced Systemic mastocytosis : a retrospective analysis of the DRST and GREM registries
| العنوان: | Allogeneic hematopoietic cell transplantation in advanced Systemic mastocytosis : a retrospective analysis of the DRST and GREM registries |
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| المؤلفون: | Lübke, Johannes, Christen, Deborah, Schwaab, Juliana, Kaiser, Anne, Naumann, Nicole, Shoumariyeh, Khalid, Jentzsch, Barbara Madlen, Sockel, Katja, Hennigs, Judith Marie, Ayuk, Francis, Stelljes, Matthias, Hilgendorf, Inken, und viele weitere |
| سنة النشر: | 2024 |
| المجموعة: | Share it - Open Access und Forschungsdaten-Repositorium der Hochschulbibliotheken in Sachsen-Anhalt |
| مصطلحات موضوعية: | ddc:610 |
| الوصف: | We identified 71 patients with AdvSM (aggressive SM [ASM], SM with an associated hematologic neoplasm [SM-AHN, e.g., acute myeloid leukemia, SM-AML], mast cell leukemia [MCL]) in two national registries (DRST/GREM) who received an allogeneic hematopoietic cell transplantation (alloHCT) performed in Germany from 1999–2021. Median overall survival (OS) of ASM/SM-AHN (n = 30, 45%), SM-AML (n = 28, 39%) and MCL ± AHN (n = 13, 19%) was 9.0, 3.3 and 0.9 years (P = 0.007). Improved median OS was associated with response of SM (17/41, 41%; HR 0.4 [0.2–0.9], P = 0.035) and/or of AHN (26/43, 60%, HR 0.3 [0.1–0.7], P = 0.004) prior to alloHCT. Adverse predictors for OS included absence of KIT D816V (10/61, 16%, HR 2.9 [1.2–6.5], P < 0.001) and a complex karyotype (9/60, 15%, HR 4.2 [1.8–10.0], P = 0.016). HLA-match, conditioning type or transplantation at centers reporting above-average alloHCTs (≥7) had no impact on OS. Taking into account competing events at years 1, 3 and 5, relapse-related mortality and non-relapse mortality rate were 15%/23%, 20%/30% and 23%/35%, respectively. Irrespective of subtype, subsequent treatment response was achieved in 13/30 (43%) patients and was highest on midostaurin/avapritinib (7/9, 78%). We conclude that outcome of alloHCT in AdvSM is more affected by disease phenotype and treatment response prior to transplant than by transplant characteristics. |
| نوع الوثيقة: | article in journal/newspaper |
| وصف الملف: | application/pdf |
| اللغة: | English |
| ردمك: | 978-1-885704-65-8 1-885704-65-8 |
| Relation: | http://dx.doi.org/10.25673/116023 |
| DOI: | 10.25673/116023 |
| الاتاحة: | https://opendata.uni-halle.de//handle/1981185920/117977 https://doi.org/10.25673/116023 |
| Rights: | https://creativecommons.org/licenses/by/4.0/ |
| رقم الانضمام: | edsbas.691B755B |
| قاعدة البيانات: | BASE |
| ردمك: | 9781885704658 1885704658 |
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| DOI: | 10.25673/116023 |