Work

Black African-American patients with primary myelofibrosis: a comparative analysis of phenotype and survival

Year: 2023

Type: article

Abstract: Racial and ethnic disparities have been documented in patients with acute myeloid leukemia through population-based surveillance, epidemiology, and end results studies in which Black patients with acute myeloid leukemia were shown to have a 12% increased risk of death compared with White patients, despite favorable cytogenetics and younger age. 1 The phenotypic or prognostic impact of the Black race on primary myelofibrosis (PMF) has not been adequately addressed.2 In a surveillance, epidemiology, and end results-Medicare database analysis of 3364 patients with myeloproliferative neoplasms (MPNs), which included 10% non-White patients with PMF, showed that survival in Black patients, compared with their non-Black counterparts, was inferior in the setting of essential thrombocythemia and polycythemia vera but not PMF. 3 A single institution retrospective study on outcome disparities in Caucasian vs non-Caucasian patients with MPN (n = 127, PMF, n = 22, non-Caucasians with PMF, n = 11), reported an increased risk of vascular complications in non-Caucasian patients with polycythemia vera and essential thrombocythemia; however, the overall survival was similar in Caucasian and non-Caucasian patients.4 In another study of 300 patients with MPN, including 14 non-White patients with PMF, survival was found to be inferior in non-White compared with White patients.5 The objectives of this study were (i) to define the clinical phenotype and genotype of Black African-American (AA) patients with PMF, and (ii) to determine the long-term survival outcomes of AA patients, in comparison with their non-AA counterparts.This multicenter study included 88 consecutive AA patients with PMF evaluated at the Mayo Clinic, Rochester MN (n = 46), Moffitt Cancer Center, Tampa, FL (n = 34), and Montefiore Medical Center, Bronx, NY (n = 8) between March 1998 and March 2022.The study patients were retrospectively recruited after institutional review board approval, with follow-ups updated in October 2022.A previously published Mayo Clinic cohort of 1266 non-Black patients with PMF (95% Caucasian) was used for comparison of phenotype and survival.6 Race has been self-reported in the current study.The diagnosis of PMF was based on conventional criteria.7 Screening for JAK2, MPL, and CALR mutations and next generation sequencing were performed in a subset of patients.8 The dynamic international prognostic scoring system (DIPSS), DIPSS-plus, mutation enhanced international prognostic score MIPSS-70 plus version 2.0 risk stratification, and unfavorable karyotype categorization were used, as previously described.[9][10][11][12] All statistical analyses considered clinical and laboratory variables obtained at the time of diagnosis.Conventional statistical methods were applied to compare clinical characteristics and survival analysis.Survival was calculated from the time of diagnosis to the last follow-up or death.JMP Pro 16.0.0software package (SAS Institute, Cary, NC) was used for statistical analysis.A total of 88 AA patients with PMF (median age 61 years; range, 19-86; 52% female) were studied.Driver mutation information was available for 63 patients and included JAK2 68%, CALR 19% (type

Source: Blood Advances

DOI: 10.1182/bloodadvances.2022009611