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).

Keywords: Acute myeloid leukemia; Allogeneic stem cell transplantation; Chemotherapy; Hypomethylating agents; Myelofibrosis; Myeloproliferative neoplasms.

Figure 1

Flowchart showing treatment options for patients with post-MPN AML. Patients are assessed for medical fitness, which can be represented with Eastern Cooperative Oncology Group (ECOG) performance status. Generally, patients with ECOG 0 - 1 can be considered fit. Patients with ECOG 2 - 3 are often considered unfit. Patients with ECOG 4 are considered frail. Most often fit patients are considered for clinical trials, however “unfit” are sometimes also included, and frail patients are rarely included. Median overall survival was gathered from previous clinical trials with ranges displayed. mOS: median overall survival; mo: months.