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Anemia is one of the major risk factors for survival in primary myelofibrosis (PMF). Current prognostic models in PMF, including the International Prognostic Scoring System (IPSS) [1], dynamic IPSS (DIPSS) [2] and DIPSS-plus [3], all list hemoglobin level (Hb) of <10 g/dl as one of their risk variables. However, the 10 g/dl Hb threshold used in these prognostic models overlooks the significant difference in Hb levels between men and women, and, also assumes similar prognostic weight between moderate and severe anemia; in regards to the latter, for example, red cell transfusion need confers an additional point of prognostic adversity in DIPSS-plus, which is independent of and in addition to what is accounted for a hemoglobin level of <10 g/dl [3]. The “dose-dependent” prognostic effect of anemia, and the effect of sex on the selection of prognostically relevant Hb thresholds, has previously been recognized in myelodysplastic syndromes (MDS) [4]. In the current study, we examined the severity-stratified and sex-specific prognostic contribution of anemia in PMF, in the context of other relevant clinical, cytogenetic and molecular risk factors. The current study was approved by the Mayo Clinic institutional review board and consent from research studies was obtained in all subjects. Consecutive cases of PMF were recruited from institutional databases of the Mayo Clinic, Rochester, MN, USA, where the 2016 World Health Organization (WHO) criteria for PMF were retrospectively applied to confirm the diagnosis [5]. Special precaution was taken to avoid inclusion of patients with post-polycythemia vera myelofibrosis; only one woman had a hemoglobin level of >16 g/dl (16.1 g/dl) and only one man had a hemoglobin level of >16.5 (16.7 g/dl); in both instances, rereview of clinical and bone marrow morphological features were fully consistent with PMF and there was no antecedent history of PV in either. Treatment was according to conventional standards utilized at the time of diagnosis. Clinical and laboratory data, including cytogenetic information, were collected at the time of initial referral to our institution. Cytogenetic analysis and reporting was done according to the International System for Human Cytogenetic Nomenclature (ISCN) criteria [6]. The recently revised system was used for cytogenetic risk stratification (Tefferi et al. personal communication, 17 December 2017; see Table 1 footnote). Survival was calculated from time of initial referral, commensurate with time of baseline data acquisition, including cytogenetic and mutation analysis. Standard statistical methods were used to determine significance of differences among groups in the distribution of continuous or nominal variables. Overall survival data were prepared by the Kaplan–Meier method and compared by the log-rank test. Cox proportional hazard regression model was applied for multivariable analysis. P value < 0.05 was considered significant. The Stat View (SAS Institute, Cary, NC, USA) statistical package was used for all calculations. Patients were stratified according to the severity of anemia: mild— Hb ≥ 10 g/dl but below sex-adjusted lower limit of normal; moderate—Hb between 8 g/dl and <10 g/dl; and severe— Hb < 8 g/dl or transfusion-dependent. Reference ranges for Hb, at our institution, were 13.5–17.5 g/dl for men and 12.0–15.5 g/dl for women. * Ayalew Tefferi tefferi.ayalew@mayo.edu

Sex and degree of severity influence the prognostic impact of anemia in primary myelofibrosis: analysis based on 1109 consecutive patients