Age older than 65 years, hemoglobin level lower than 100 g/L (10 g/dL), white blood cell count greater than 25 x 10(9)/L, peripheral blood blasts 1% or higher, and constitutional symptoms have been shown to predict poor survival in primary myelofibrosis (PMF) at diagnosis. To investigate whether the acquisition of these factors during follow-up predicts survival, we studied 525 PMF patients regularly followed. All 5 variables had a significant impact on survival when analyzed as time-dependent covariates in a multivariate Cox proportional hazard model and were included in 2 separate models, 1 for all patients (Dynamic International Prognostic Scoring System [DIPSS]) and 1 for patients younger than 65 years (age-adjusted DIPSS). Risk factors were assigned score values based on hazard ratios (HRs). Risk categories were low, intermediate-1, intermediate-2, and high in both models. Survival was estimated by the HR. When shifting to the next risk category, the HR was 4.13 for low risk, 4.61 for intermediate-1, and 2.54 for intermediate-2 according to DIPSS; 3.97 for low risk, 2.84 for intermediate-1, and 1.81 for intermediate-2 according to the age-adjusted DIPSS. The novelty of these models is the prognostic assessment of patients with PMF anytime during their clinical course, which may be useful for treatment decision-making.
"These discrepancies may rely on the clinical characteristics of the patients entered in the two studies, since the PMF patients described by Huang et al. were younger, had a lower WBC count and a higher Hb level. The prognostic power of anemia, has been strongly restated precisely by the new dynamic international prognostic scoring system (DIPSS) and by its more recently implementation into the DIPSS-plus that assigned to patients with Hb <10 g/dL the highest score of 2 . In this latter study, patients who acquired a high-risk score during the follow-up have a notable 24.9 fold higher risk of develop LT compared to those patients who continue to fit in low risk categories. "
[Show abstract][Hide abstract] ABSTRACT: To predict leukemic transformation (LT) we evaluated easily detectable diagnostic parameters in 338 patients with primary myelofibrosis (PMF) followed in the Latium region (Italy) between 1981 and 2010. Forty patients (11.8%) progressed to leukemia, with a resulting 10-year leukemia-free survival (LFS) rates of 72%. Hb (≤ 10 gr/dL), and circulating blasts (≥ 1%) were the only two independent prognostic for LT at the multivariate analysis. Two hundred-fifty patients with both the two parameters available were grouped as follows: low risk (none or one factor) = 216 patients; high risk (both factors) = 31 patients. The median LFS times were 269 and 45 months for the low and high-risk groups, respectively (P < 0001). The LT predictive power of these two parameters was confirmed in an external series of 270 PMF patients from Tuscany, in whom the median LFS was not reached and 61 months for the low and high risk groups, respectively (P<.0001). These results establish anemia and circulating blasts, two easily and universally available parameters, as strong predictors of LT in PMF and may help to improve prognostic stratification of these patients particularly in countries with low resources where more sophisticated molecular testing is unavailable.
Leukemia Research 01/2015; 39(3). DOI:10.1016/j.leukres.2015.01.001 · 2.35 Impact Factor
"PMF was diagnosed according to the World Health Organization criteria . Dynamic International Prognostic Scoring System (DIPSS) and DIPSS-plus scores were assigned to each patient as previously described   . Demographic and clinical information at the time of presentation to MDACC, including medical history, results of physical examination, complete blood count and blood chemistry, and assessment of bone marrow aspiration/biopsy, were extracted from patient records. "
"The assessments of Bcr/Abl rearrangement and JAK2 V617F mutation were both negative. According to 2008 WHO criteria , a diagnosis of PMF was therefore made, with an International Prognostic Score System (IPSS)  score of 2 (intermediate 2 risk) and a Dynamic International Prognostic Score System (DIPSS)  score again of 2 (intermediate 1 risk). After few months of clinical observation, the patient progressively developed extreme leukocytosis (WBC 100 × 10 9 /L), mild thrombocytopenia (Plt 120 × 10 9 /L), and worsening of the normocytic anemia (Hb 10.0 g/dL). "
[Show abstract][Hide abstract] ABSTRACT: Primary myelofibrosis (PMF) is a myeloproliferative neoplasm frequently complicated by transfusion dependent anemia. Both anemia and transfusion-dependence are associated with a poor outcome, at least in part because of toxic effects of iron overload (IOL). Iron-chelating therapy (ICT) is increasingly used in order to prevent IOL in this setting. Here, we describe the case of a 73-year-old man affected by PMF and severe transfusion-dependent anemia who experienced a dramatic erythroid response after being treated with deferasirox to prevent IOL.
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