Population-based incidence and survival figures in essential thrombocythemia and agnogenic myeloid metaplasia: An Olmsted county study, 1976-1995

Abstract

To provide basic information about occurrence and outcome of essential thrombocythemia (ET) and agnogenic myeloid metaplasia (AMM), we used the Rochester Epidemiology Project medical record linkage system for residents of Olmsted County, Minnesota. We identified all residents who were diagnosed with ET or AMM from 1976 to 1995. Community inpatient and outpatient medical records were reviewed to verify the diagnosis of ET or AMM, and patients were followed passively through their medical records to determine the outcome after diagnosis. We identified 39 cases of ET and 21 of AMM, with age- and sex-adjusted incidence rates of 2.53 and 1.46 cases/100,000 population annually, respectively. The respective median ages at diagnosis were 72 and 67 years. The female-to-male ratios were 1.8 and 1.6 for ET and AMM, respectively, and when adjusted for age, there was no difference in risk. The median follow-up period was 62.9 months for ET and 33.2 months for AMM. Five- and 10-year survivals were 74.4% and 61.3%, respectively, for ET and were significantly lower than expected for age-matched controls (P = 0.012). Prognosis was worse for AMM, with a median progression time of 7 months and a 3-year survival of 52.4%. This was significantly worse than for age-matched controls (P < 0.001). This study provides population-based incidence and comparative survival figures in ET and AMM.

One Thousand Patients With Primary Myelofibrosis: The Mayo Clinic Experience

“…In a previous population-based study, the median age at diagnosis was similar at 67 years. 34 At time of referral, only 13% of the patients were younger than 50 years and 31% were younger than 60 years (data not shown). The corresponding figures for patients seen 35 and pomalidomide.…”

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

“…Primary myelofibrosis, also known as idiopathic myelofibrosis or myelofibrosis with myeloid metaplasia, is a rare disease 19,20 usually affecting elderly people. Median survival ranges from 4 to 5.5 years in modern series [6][7][8][9][10][11][12][13][14] (Figure 1).…”

“…[10][11][12]30 Age at presentation is another variable linked to the prognosis of PMF, 7,9,10,12-14 with younger subjects surviving longer. 19,20 Although the latter statement applies to the minority of individuals 55-year old or younger at diagnosis, in patients with age over 55 years, the prognostic influence of advanced age is less clear, as at multivariate analysis, the poorer prognosis of older patients was maintained in two studies, 7,14 but not in the majority. 9,[11][12][13] Male sex was associated with worse prognosis at univariate study in the Lille series, but lost significance at multivariate level.…”