Primary myelofibrosis: risk stratification by IPSS identifies patients with poor clinical outcome

Abstract

OBJECTIVES:To evaluate whether risk scores used to classify patients with primary myelofibrosis and JAK-2 V617F mutation status can predict clinical outcome.METHODS:A review of clinical and laboratory data from 74 patients with primary myelofibrosis diagnosed between 1992 and 2011. The IPSS and Lille scores were calculated for risk stratification and correlated with overall survival.RESULTS:A V617F JAK2 mutation was detected in 32 cases (47%), with no significant correlation with overall survival. The patients were classified according to the scores: Lille - low, 53 (73.%); intermediate, 13 (18%); and high, 5 (7%); and IPSS – low, 15 (26%); intermediate-1, 23 (32%); intermediate-2, 19 (26%); and high, 15 (31%). Those patients presenting a higher risk according to the IPSS (high and intermediate-2) had a significantly shorter overall survival relative to the low risk groups (intermediate-1 and low) (p = 0.02).CONCLUSIONS:These results emphasize the importance of the IPSS prognostic score for risk assessment in predicting the clinical outcome of primary myelofibrosis patients.

Primary Idiopathic Myelofibrosis: Clinico-Epidemiological Profile and Risk Stratification in Pakistani Patients

“…In one prior Brazilian study; 73% had low risk; intermediate in 18% and high risk disease in only 7% (Benites et al, 2013). Compared with this majority of our patients were in intermediate risk which is in consensus with study reported by German group; 56% and 23% in intermediate and high risk respectively (Alchalby et al, 2012).…”

“…The two adverse prognostic factors, namely Hb <10 g/dL and WBC <4 or >30x10 3 /L, was able to separate patients in three groups; with low (0 factor), intermediate (1 factor) and high (2 factors) (Benites et al, 2013).…”

Real-World Assessment of Clinical Outcomes in Patients with Lower-Risk Myelofibrosis Receiving Treatment with Ruxolitinib

“…In our study sample, we also found that more than half (53%) of patients with intermediate-1-risk MF had moderate to severe splenomegaly (palpable spleen > 10 cm) at MF diagnosis. Two previous medical record reviews [ 22 , 23 ] also indicated the possibility of a considerable rate of moderate to severe splenomegaly, even in patients with lower-risk MF. These studies, however, examined pooled cohorts of patients with both lower-risk (low and intermediate-1 risk) and higher-risk (intermediate-2 and high risk) patients and did not stratify splenomegaly frequency by risk category at diagnosis.…”

Safety and outcome of allogeneic stem cell transplantation in myelofibrosis

“…It should be mentioned that >90% of JAK2 ‐ positive patients eradicated or decreased the clone after transplant. Published data regarding the role of the mutation have demonstrated conflictive results . Although the presence of JAK2 V617F mutation before transplantation is found to predict a favorable outcome , patients who remained JAK2 positive at 6 months after allo‐HSCT had a significantly greater risk of progression .…”

“…It should be mentioned that >90% of JAK2-positive patients eradicated or decreased the clone after transplant. Published data regarding the role of the mutation have demonstrated conflictive results (13).…”