Myelofibrosis with myeloid metaplasia: clinical and haematological parameters predicting survival in a series of 133 patients

Abstract

The prognostic value of 12 clinical and haematological parameters, recorded at diagnosis, in myelofibrosis with myeloid metaplasia (MMM) was retrospectively analysed in a consecutive series of 133 patients followed for a minimum of 60 months. Multivariate analysis showed that the following features were associated with a significantly shorter survival: (1) short period of time (less than 13 months) between first symptoms and diagnosis; (2) anaemia (haemoglobin less than 10 g/dl); (3) leucocyte count greater than 12 x 10(9)/l; (4) peripheral blood granulocyte precursors greater than 10%. Age, splenectomy and percentage of peripheral blood metamyelocytes were found significantly to affect survival only from univariate analysis, whereas sex, size of spleen, thrombocytopenia and thrombocytosis were of no prognostic significance. These data suggest that a more intensive chemotherapy might be useful for younger patients with bad prognostic factors at diagnosis.

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].…”

Dermatomyositis and myelodysplastic syndrome with myelofibrosis responding to methotrexate therapy

“…A few months before the onset of DM, he was also diagnosed as having MDS with myelofibrosis on the basis of anemia, the appearance of immature cells in peripheral blood, cellular atypism, bone marrow biopsy findings, and the absence of any noticeable splenomegaly. Splenomegaly is reported to be present in 85-99% of primary myelofibrosis (PMF) patients [11,12], but our patient did not show any signs of splenomegaly. He was, therefore, considered to have MDS with myelofibrosis or MDS/MPD rather than PMF.…”

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.…”

“…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.…”