Acute Myeloid Leukemia Following Myeloproliferative Neoplasms: A Review of What We Know, What We Do Not Know, and Emerging Treatment Strategies

Abstract

Acute myeloid leukemia (AML) arising from myeloproliferative neoplasms (MPNs) represents a small subtype of secondary AML (sAML). This entity is well known to be associated with poor responses to available treatment options and dismal outcomes. To date, there are no standardized treatment options and there has been very little therapeutic advancement in recent years. This is a stark contrast to other subsets of AML for which there have been significant advances in therapeutic approaches, especially for patients with targetable mutations. We aim to focus our review on the incidence, risk factors for leukemogenesis, pathogenesis, molecular landscape, and emerging therapeutic options in post-myeloproliferative neoplasm acute myeloid leukemia (post-MPN AML).

Mesenchymal stromal cells in myeloid malignancies: Immunotherapeutic opportunities

“…This set of neoplasms includes Polycythemia vera (PV) , characterized by an elevated red cell count, often accompanied by thrombocytosis; Essential thrombocythemia (ET) , diagnosed by megakaryocyte expansion and increased platelet count; and Primary myelofibrosis (PMF): Which display extramedullary hematopoiesis, splenic enlargement, and a fibrotic BM [ 48 , 56 , 58 , 59 ]. ET or PV can progress to myelofibrosis with bone marrow failure or to secondary acute leukemia [ 60 , 61 ]. The incidence of PV and ET are approximately 0.5–4.0 and 1.1–2.0 cases per 100,000 person-years, respectively, and comparable survival degrees.…”

Genomic alterations in blast phase of BCR::ABL1‐negative myeloproliferative neoplasms

“…Total 13%-16% 28,29 ET 5% 29 PV 16%-25% 22,29 PMF 10%-17% 20,29 Total 19%-28% 12,13 ET 16% 14 PV 17%-27% 14,22 PMF 33% 14 Negative mRNA splicing regulator SRSF2 ET 2% 15,19 PV 3% 15,19 PMF 8%-22% 15,19 Total 13%-19% [12][13][14] ET 11% 14 PV 0% 14 PMF 27%-50% 14,17 Negative U2AF1 ET 1% 15 PV 1%-7% 15,19 PMF 15% 17 PMF (U2AF1 Q157) 8% 17 Total 5%-6% 12,13 PMF 15% 17 PMF (U2AF1 Q157) 10% 17 Negative on PMF and ET Cell cycle checkpoints regulator TP53 Total 2%-5% 13,40 ET 2%-6% 15,19 PV 1%-6% 15,19,22 PMF 1%-5% 15,19 Total 16%-36% 13,14,34 ET 53% 14 PV 50%-55% 14,22<...…”