Life expectancy of patients with chronic nonleukemic myeloproliferative disorders

Abstract

This study determines, within the frame of current therapeutic possibilities, the impact of chronic nonleukemic myeloproliferative disorders on expected survival. The survival data for 1067 patients (454 with polycythemia vera, 247 with essential thrombocythemia, and 366 with idiopathic myelofibrosis) were collected from 38 Spanish institutions. The actuarial survival probability of each group of patients was compared with that of the age-matched and sex-matched control population. The survival of the patients with polycythemia vera and essential thrombocythemia did not differ from that of the control population (P = 0.92 and, 0.22, respectively), whereas the survival of the patients with idiopathic myelofibrosis was strikingly reduced with respect to the control population (P = 0.0000000007). Thus, in terms of survival, current therapeutic procedures may be considered as quite satisfactory in patients with polycythemia vera and essential thrombocythemia. On the other hand, due to poor survival of patients with idiopathic myelofibrosis, new therapeutic approaches for this condition are clearly needed.

Follow‐up examinations including sequential bone marrow biopsies in essential thrombocythemia (ET): A retrospective clinicopathological study of 120 patients

“…On the other hand, BM biopsies are easy to perform and evaluation of histological features (reading of slides) can be properly accomplished by both the hematopathologist and the interested clinician during a clinicopathological conference meeting. Contrasting IMF [28,[44][45][46][47][48] patients with ET have a nearnormal life expectancy which probably depends on the low rate of leukemic transformation [2,15,16,21,44,48,49]. However, persuasive evidence has been produced by several clinical studies that risk stratification in ET is based primarily on factors related to thrombohemorrhagic complications and high age (>60 years) and especially a previous history of thrombosis [2][3][4][49][50][51].…”

Life expectancy and prognostic factors in the classic BCR/ABL-negative myeloproliferative disorders

“…When the survival of PMF patients has been compared with that of age-and sexmatched individuals from the general population, a 31% reduction in life expectancy has been observed. 15 Although due to the increasing frequency of routine laboratory studies, there is a trend toward earlier PMF diagnosis, comparison of the survival of patients diagnosed during different time periods indicates that a possible earlier diagnosis would not translate into a longer survival. 21 Main causes of death in PMF are evolution to acute leukaemia (observed in 20% of cases at 10 years of diagnosis), infection and bleeding secondary to bone marrow failure, portal hypertension or hepatic failure secondary to hepatic/splenoportal vein thrombosis or myeloid metaplasia of the liver, thromboses in other territories and heart failure.…”

“…[15][16][17][18]20,48,49 A study on 831 patients with PV and ET followed for a median time of about 10 years provided evidence that life expectancy of PV patients is reduced when compared with that of the general population, whereas the survival of patients with ET is not significantly shortened. 18 A study from Mayo Clinic including 322 patients with ET followed for a median time of 13.6 years confirmed these data on survival in the first decade of disease and showed that survival became significantly worse thereafter.…”

“…With regard to the survival, PMF is associated with a substantial reduction in life expectancy, [6][7][8][9][10][11][12][13][14] and ET affects more the patients' quality of life than their survival, whereas PV is associated with both a substantial morbidity and a certain reduction in the patients' life expectancy. [15][16][17][18] Because of this, prognostic studies in the MPDs have been carried out primarily in PMF. In the last years, however, attention is increasingly being paid to the identification of prognostic factors in ET and PV, primarily focused on the risk of thrombosis.…”

Role of blood cells dynamism on hemostatic complications in low-risk patients with essential thrombocythemia

“…Patients with ET have a relatively benign prognosis [2], but thrombotic and hemorrhagic events may indeed occur over time, leading to severe morbidity [3][4][5]. Previous studies by Cortelazzo et al [6][7][8] showed that age [60 years and previous thrombotic or hemorrhagic events are strongly associated with a higher risk of developing further events.…”