Allogeneic hematopoietic cell transplantation in advanced Systemic mastocytosis: a retrospective analysis of the DRST and GREM registries
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...
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| Hauptverfasser: | , , , , , , , , , , , , , , , , |
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| Dokumenttyp: | Article (Journal) |
| Sprache: | Englisch |
| Veröffentlicht: |
06 March 2024
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| In: |
Leukemia
Year: 2024, Jahrgang: 38, Heft: 4, Pages: 810-821 |
| ISSN: | 1476-5551 |
| DOI: | 10.1038/s41375-024-02186-x |
| Online-Zugang: | Resolving-System, kostenfrei, Volltext: https://doi.org/10.1038/s41375-024-02186-x Verlag, kostenfrei, Volltext: https://www.nature.com/articles/s41375-024-02186-x Resolving-System, kostenfrei: https://doi.org/10.25673/116023 
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| Verfasserangaben: | Johannes Lübke, Deborah Christen, Juliana Schwaab, Anne Kaiser, Nicole Naumann, Khalid Shoumariyeh, Madlen Jentzsch, Katja Sockel, Judith Schaffrath, Francis A. Ayuk, Matthias Stelljes, Inken Hilgendorf, Elisa Sala, Jennifer Kaivers, Stefan Schönland, Christoph Wittke, Bernd Hertenstein, Markus Radsak, Ulrich Kaiser, Valeska Brückl, Nicolaus Kröger, Tim H. Brümmendorf, Wolf-Karsten Hofmann, Stefan Klein, Edgar Jost, Andreas Reiter, Jens Panse |
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| 245 | 1 | 0 | |a Allogeneic hematopoietic cell transplantation in advanced Systemic mastocytosis |b a retrospective analysis of the DRST and GREM registries |c Johannes Lübke, Deborah Christen, Juliana Schwaab, Anne Kaiser, Nicole Naumann, Khalid Shoumariyeh, Madlen Jentzsch, Katja Sockel, Judith Schaffrath, Francis A. Ayuk, Matthias Stelljes, Inken Hilgendorf, Elisa Sala, Jennifer Kaivers, Stefan Schönland, Christoph Wittke, Bernd Hertenstein, Markus Radsak, Ulrich Kaiser, Valeska Brückl, Nicolaus Kröger, Tim H. Brümmendorf, Wolf-Karsten Hofmann, Stefan Klein, Edgar Jost, Andreas Reiter, Jens Panse |
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| 520 | |a 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. | ||
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