| Author(s) |
|
| Abstract |
|
To review the updated trends of national practice and outcomes in transplanting myelofibrosis, we retrospectively evaluated 142 patients who underwent allogeneic hematopoietic stem cell transplantation for primary (n=94) or secondary (n=48) myelofibrosis (MF) at Australian/New Zealand transplant centers between 2006 and 2017. Median follow-up was 51.8 months (range: 3.1-148). Median age at allo-SCT was 56 years (range: 26-69). Fifty-two percent had HLA-identical sibling donors and 45% had matched unrelated donors (UD). Conditioning was predominantly reduced intensity (83%). Before transplant, 16% had splenectomy or splenic irradiation and 54 patients (38%) received JAK inhibitors. JAK2 mutation testing was performed in 66.9% of patients whilst other mutations CALR, MPL, ASXL1, SRSF2, U2AF1Q57, EZH2 and IDH1/2 were rarely tested (1.4-8.4%). Only 4.2% of patients had next generation sequencing mutation analysis. Median time to neutrophil engraftment was 19 days (range: 10-43) and median time to platelet engraftment was 27 days (range: 13-230). The cumulative incidences of grade II-IV acute graft-versus-host disease (GvHD) were 21.4% at 100 days and that of extensive chronic GvHD at 5 years was 18.1%. Overall survival (OS) was 67% at 1 year and 57% at 5 years. GvHD-free, relapse-free survival was 54% at 1 year and 42% at 5 years. The cumulative incidence of non-relapse mortality (NRM) was 16% at 100 days and 25% at 1 year. In multivariate analysis, age ≥ 65 years and use of an UD were significant unfavourable risk factors for OS and NRM. Use of an UD increased the incidence of acute GvHD whereas antithymocyte globulin/ alemtuzumab lowered the risk of both acute GvHD and chronic GvHD. Pretransplant splenectomy/splenic irradiation had a positive influence on time to engraftment. There have been no improvements in MF allo-SCT outcomes in Australasia in the last decades with low uptake of molecular genomic technology due to limited funded access. |
|
|
