Primary myelofibrosis: A detailed statistical analysis of the clinicopathological variables influencing survival

Abstract

In the present study we analyzed the prognostic significance of several clinical, hematological, and histological parameters recorded at diagnosis in a consecutive series of 72 patients with primary myelofibrosis (PMF). Univariate analysis showed that the most significant indicators of poor survival were the following: age greater than 60, splenomegaly, anemia (hemoglobin > 10 g/dl), leukopenia (WBC < 4 x 10(9)/l or leukocytosis > 14 x 10(9)/l), and any of these histological features: adipose tissue and megakaryocyte reduction, prominent osteoblastic rims along the trabecular bone, presence of peritrabecular megakaryocytes (Mk), absence of normal or giant Mk. The multivariate analysis showed that only the level of hemoglobin and the presence of both normal Mk and fever independently influenced the prognosis. These parameters were used to set up a prognostic scoring system, allowing a feasible prognosis to be made for each patient at the time of diagnosis and identifying those patients in urgent need of new therapeutic approaches.

Prodromal myeloproliferative neoplasms: The 2008 WHO classification

“…This difference may be due to a longer observation time (median follow-up: 6.6 years, total of 5,650 person years) and very important, early diagnosis was facilitated by strictly performing a BM biopsy at onset of a suspected MPN with presentation of relevant clinical features. Comparable with advanced stage PMF [22,102,128,[130][131][132][133][134][135][136][138][139][140][141], degree of anemia (initial hemoglobin level), age at diagnosis, presence of peripheral blasts and platelet as well as leukocyte counts were of significant predictive value [102,128,135,138]. These results are in keeping with the assumption that in prodromal PMF according to multivariate risk analysis features indicating expansion of disease with involvement of extramedullary organs or myeloid metaplasia exerts the most important impact on prognosis [102,128,135].…”

“…Altogether a stepwise, however, unpredictable evolution of the disease process in PMF associated with correspondingly expressed clinical data has to be realized [111,114]. An extreme heterogeneity of survival patterns has been reported in larger series of patients with PMF ranging between 3.5 and 10 years [22,102,128,[130][131][132][133][134][135][136][137][138][139][140][141]. However, only a few of these studies considered the full spectrum of disease manifestation including prodromal stages [108,128,135,138].…”

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

“…Median survival ranges from 4 to 5.5 years in modern series [6][7][8][9][10][11][12][13][14] (Figure 1). 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.…”

“…[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.…”

“…The recent discovery of the mutation V617F in the JAK2 gene in a high proportion of MPD patients [1][2][3][4] has given biological support to the early notion by Dameshek 5 placing the three disorders within the same group. 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.…”