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Lübke, Johannes [Author]; Christen, Deborah [Author]; Schwaab, Juliana [Author]; Kaiser, Anne [Author]; Naumann, Nicole [Author]; Shoumariyeh, Khalid [Author]; Jentzsch, Barbara Madlen [Author]; Sockel, Katja [Author]; Hennigs, Judith Marie [Author]; Ayuk, Francis [Author]; Stelljes, Matthias [Author]; Hilgendorf, Inken [Author]; Schönland, Stefan [Author]; Wittke, Christoph [Author]

Allogeneic hematopoietic cell transplantation in advanced Systemic mastocytosis

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  • Media type: E-Article
  • Title: Allogeneic hematopoietic cell transplantation in advanced Systemic mastocytosis : a retrospective analysis of the DRST and GREM registries
  • Contributor: Lübke, Johannes [Author]; Christen, Deborah [Author]; Schwaab, Juliana [Author]; Kaiser, Anne [Author]; Naumann, Nicole [Author]; Shoumariyeh, Khalid [Author]; Jentzsch, Barbara Madlen [Author]; Sockel, Katja [Author]; Hennigs, Judith Marie [Author]; Ayuk, Francis [Author]; Stelljes, Matthias [Author]; Hilgendorf, Inken [Author]; Schönland, Stefan [Author]; Wittke, Christoph [Author]
  • Published: 06 March 2024
  • Published in: Leukemia ; 38(2024), 4, Seite 810-821
  • Language: English
  • DOI: 10.1038/s41375-024-02186-x; 10.25673/116023
  • Identifier:
  • Keywords: Myeloproliferative disease ; Translational research
  • Origination:
  • Footnote:
  • Description: 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.
  • Access State: Open Access
  • Rights information: Attribution (CC BY)