Luebke, Johannes and Christen, Deborah and Schwaab, Juliana and Kaiser, Anne and Naumann, Nicole and Shoumariyeh, Khalid and Jentzsch, Madlen and Sockel, Katja and Schaffrath, Judith and Ayuk, Francis A. and Stelljes, Matthias and Hilgendorf, Inken and Sala, Elisa and Kaivers, Jennifer and Schoenland, Stefan and Wittke, Christoph and Hertenstein, Bernd and Radsak, Markus and Kaiser, Ulrich and Brueckl, Valeska and Kroeger, Nicolaus and Bruemmendorf, Tim H. and Hofmann, Wolf-Karsten and Klein, Stefan and Jost, Edgar and Reiter, Andreas and Panse, Jens (2024) Allogeneic Hematopoietic Cell Transplantation in Advanced Systemic Mastocytosis: A retrospective analysis of the DRST and GREM registries. LEUKEMIA, 38 (4). pp. 810-821. ISSN 0887-6924, 1476-5551
Full text not available from this repository. (Request a copy)Abstract
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.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | ACUTE MYELOID-LEUKEMIA; METHYLTRANSFERASE GENE EZH2; MYELODYSPLASTIC SYNDROME; BONE-MARROW; MOUSE MODELS; NUCLEOPORIN NUP98; XENOGRAFT MODEL; MUTATIONS; MICE; ENGRAFTMENT; |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Innere Medizin III (Hämatologie und Internistische Onkologie) |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 20 Aug 2025 08:03 |
| Last Modified: | 20 Aug 2025 08:03 |
| URI: | https://pred.uni-regensburg.de/id/eprint/65583 |
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