Leukemic transformation in myelofibrosis with myeloid metaplasia: a single-institution experience with 91 cases
Open accessAbstract
Among 2333 consecutive patients with myelofibrosis with myeloid metaplasia (MMM) seen at our institution, 91 fulfilled the World Health Organization (WHO) criteria for leukemic transformation (LT). All episodes of LT were myeloid in origin (acute myeloid leukemia [AML]) with all French-American-British (FAB) subtypes represented except M3; the most frequent subtypes were M7 (25.4%), M0 (22.4%), and M2 (17.9%). Cytogenetic studies during LT were available in 56 patients and revealed a clonal abnormality in 51 (91%): 30 patients had complex karyotype, 2 had core-binding factor gene lesions, and 18 had abnormalities of chromosome 5 or 7. Karyotypic evolution was documented in the majority of the patients in whom serial analysis was possible. In general, LT was fatal in 98% of the cases after a median of 2.6 months (range, 0-24.2 months). Twenty-four patients received AML-like induction chemotherapy that resulted in no complete remission: 41% reverted into chronic-phase disease and the incidence of treatment-related mortality was 33%. The remaining 67 patients received either supportive care alone (48 patients) or low-intensity chemotherapy (19 patients). Overall, survival was similarly poor in all 3 treatment categories. The outcome of LT in MMM with current therapies is dismal and either supportive care alone or appropriate clinical trials should be considered.
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TP53 multihit HSPCs clustered separately from TP53-WT pre-LSCs in two distinct populations with enrichment of LSC and erythroid-associated transcription, respectively, and a differentiation trajectory toward the erythroid-biased population.
“Integration of single-cell transcriptomes and diffusion map analysis of HSPCs from TP53-sAML patients showed that TP53 multihit HSPCs clustered separately from TP53-WT pre-LSCs in two distinct populations with enrichment of LSC and erythroid-associated transcription, respectively (Fig. 2a and Supplementary Table 3), and a differentiation trajectory toward the erythroid-biased population (Fig. 2b),”
From Single-cell multi-omics identifies chronic inflammation as a driver of TP53-mutant leukemic evolution (2023) · cites this paper as [22]
The Methods and karyotypic-changes sections describe bone marrow histology and cytogenetic analysis in patients with myelofibrosis; no page numbers are available in our copy. Searches for TP53 multihit HSPCs, single-cell transcriptomes, diffusion maps, and an erythroid-biased differentiation trajectory found no passage addressing the claim. (LLM, conf=0.78)
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Rodriguez-Meira, A., Norfo, R., Wen, S., Chédeville, A. L., Rahman, H., O’Sullivan, J., Wang, G., Louka, E., Kretzschmar, W. W., Paterson, A., et al. (2023) · Nature Genetics · doi:10.1038/s41588-023-01480-1 · Cited by 158 · see citing works
- Authors
- Mesa, R. A., Li, C.-Y., Ketterling, R. P., Schroeder, G. S., Knudson, R. A., & Tefferi, A.
- Affiliations
- Mayo Clinic (US)
- Venue
- Blood
- Publisher
- American Society of Hematology
- Published
- 2005-02-01
- Volume
- 105
- Issue
- 3
- Pages
- 973-977
- ISSN
- 0006-4971 (ISSN-L)
- 1528-0020
- OpenAlex
- W2056116861
- Cited by
- 389
- References
- 0
- Citations by year
- 2026: 6 · 2025: 5 · 2024: 16 · 2023: 15 · 2022: 21 · 2021: 13 · 2020: 29 · 2019: 18 · 2018: 19 · 2017: 15 · 2016: 22 · 2015: 29 · 2014: 27 · 2013: 29 · 2012: 25
- FWCI
- 5.69
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Blood (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Genetics › Myeloproliferative Neoplasms: Diagnosis and Treatment
- Health Sciences › Medicine › Hematology › Acute Myeloid Leukemia Research
- Health Sciences › Medicine › Emergency Medicine › Hematological disorders and diagnostics
- Keywords
- myelofibrosis with myeloid metaplasia, leukemic transformation, acute myeloid leukemia, FAB subtypes, chromosomal abnormalities, complex karyotype, karyotype evolution, non-relapse mortality, supportive care, low-intensity chemotherapy, survival
- MeSH terms
- Adult, Aged, Aged, 80 and over, Cell Transformation, Neoplastic, Female, Humans, Leukemia, Male, Middle Aged, Myelodysplastic Syndromes, Reference Values, Leukemia, Myelogenous, Chronic, BCR-ABL Positive, Treatment Outcome, Age of Onset, Primary Myelofibrosis
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