Title | Clinicopathologic and genetic characterization of nonacute -mutated myeloid neoplasms. |
Publication Type | Journal Article |
Year of Publication | 2019 |
Authors | Patel SS, Ho C, Ptashkin RN, Sadigh S, Bagg A, Geyer JT, Xu ML, Prebet T, Mason EF, Seegmiller AC, Morgan EA, Steensma DP, Winer ES, Wong WJ, Hasserjian RP, Weinberg OK |
Journal | Blood Adv |
Volume | 3 |
Issue | 9 |
Pagination | 1540-1545 |
Date Published | 2019 05 14 |
ISSN | 2473-9537 |
Keywords | Adult, Aged, Aged, 80 and over, DNA (Cytosine-5-)-Methyltransferases, Female, Humans, Male, Middle Aged, Mutation, Myelodysplastic Syndromes, Nuclear Proteins, Prognosis, Proportional Hazards Models, Protein Tyrosine Phosphatase, Non-Receptor Type 11, Survival Rate, Tumor Suppressor Protein p53 |
Abstract | -mutated myeloid neoplasms ( MNs) with <20% blood or bone marrow blasts are rare and have been previously shown in limited case series to exhibit an aggressive clinical course. We assembled the largest cohort of MN cases to date (n = 45) and compared it with MN (n = 95) and de novo acute myeloid leukemia (AML; n = 119) patients. Compared with MN, MN were associated with younger age ( = .007), a normal karyotype ( < .0001), more frequent mutations involving ( = .01) and ( = .03), and fewer involving ( = .003), ( = .0004), and ( = .02). Mutations involving or () ( = .007) and (internal tandem duplication, < .0001; noninternal tandem duplication, = .01) were less frequent in MN than in AML. In multivariable analyses performed in patients with myelodysplastic syndrome only, total mutation count (hazard ratio [HR], 1.3; = .05), mutation (HR, 3.6; = .02), mutation (HR, 5.2; = .01), and higher International Prognostic Scoring System-R score (HR, 1.7; = .0003) were independently associated with shorter overall survival, whereas stem cell transplant conferred a favorable effect (HR, 0.1; < .0001). These data suggest that MN are biologically distinct from MN. Similar to AML, patients with -mutated myelodysplastic syndrome may benefit from more intensive therapeutic regimens. |
DOI | 10.1182/bloodadvances.2019000090 |
Alternate Journal | Blood Adv |
PubMed ID | 31085507 |
PubMed Central ID | PMC6517660 |
Grant List | P30 CA008748 / CA / NCI NIH HHS / United States UL1 TR001863 / TR / NCATS NIH HHS / United States |
Related Faculty:
Julia Geyer, M.D. Sanjay Patel, M.D., MPH