Prognostic factors in myelofibrosis

Manoharan, A.; Smart, Richard C.; Pitney, W. R.

doi:10.3109/00313028209092127

Cited by 39 publications

(42 citation statements)

References 13 publications

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“…The median survival of 5 years registered in the present series is in accordance with those reported in other recent studies, which range from 3·5 to 5·7 years (Manoharan et al, 1982;Barosi et al, 1988;Visani et al, 1990;Rupoli et al, 1994;Thiele et al, 1996;Dupriez et al, 1996). The often conflicting results of the prognostic studies in IM can probably be explained through differences in diagnostic criteria and patient selection.…”

Section: Discussionsupporting

confidence: 93%

“…With regard to anaemia, there is universal agreement on its prognostic importance in IM (Manoharan et al, 1982;Silverstein, 1985;Varki et al, 1983;Njoku et al, 1983;Barosi et al, 1988; Visani et al (1990) (B) Actuarial survival curves of the patients classified according to the Lille prognostic score (Dupriez et al, 1996). Hasselbach, 1990;Visani et al, 1990;Rupoli et al, 1994;Thiele et al, 1996;Dupriez et al, 1996).…”

Section: Discussionmentioning

confidence: 99%

“…This fact has stimulated the interest in the study of prognostic factors in this disease, to identify subgroups of patients with different outcome. In this sense, some authors have called attention to the possible existence of two prognostic populations in IM: 'short-lived' and 'long-lived' patients (Ward & Block, 1971;Manoharan et al, 1982). In order to confirm the latter notion, more studies on large IM series are required.…”

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confidence: 99%

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Identification of ‘short‐lived’ and ‘long‐lived’ patients at presentation of idiopathic myelofibrosis

et al. 1997

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To contribute to a better knowledge of the prognosis of idiopathic myelofibrosis (IM), the prognostic value of the presenting features in 106 patients diagnosed with IM at a single institution during a 21‐year period was retrospectively analysed. Median survival was 59.4 months (95% CI 40.7–75.4). Using univariate analysis, age < 64 years, constitutional symptoms (fever, night sweats, weight loss), Hb < 10 g/dl, circulating blasts (≫1%), and serum LDH > 3 times upper normal level were associated with a significantly shorter survival; male sex, platelet count <100×109/l, blood percentage of immature granulocytes (excluding blasts), low cholesterol levels and advanced marrow histological stage had borderline significance. Using multivariate study, only age > 64 years, constitutional symptoms, Hb < 10 g/dl, and circulating blasts retained their prognostic relevance. The latter three variables confirmed their predictive value in patients above and below the series median age, and were able to identify two groups of patients: a low‐risk group of 67 patients with none or one bad prognostic factor, in whom IM had an indolent course (median survival 98.8 months, 95% CI 68.7–127.6), and a high‐risk group, including 39 patients with two or three factors, with a more aggressive disease (median survival 20.6 months, 95% CI 10–28.2). Finally, the application of two recently proposed scoring systems (in which three prognostic groups are considered) was unable to separate intermediate‐ from high‐risk patients.

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Section: Discussionsupporting

confidence: 93%

Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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Identification of ‘short‐lived’ and ‘long‐lived’ patients at presentation of idiopathic myelofibrosis

et al. 1997

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“…The clinical course and prognosis are also extremely different; some patients live in good condition for years, while others show a rapidly worsening course [5,17,24,27,30,32,34,36].…”

Section: Introductionmentioning

confidence: 99%

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

et al. 1994

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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.

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“…A wealth of data has been accumulated in recent years about the extreme heterogeneity of survival patterns in IMF [11] when univariate [1,17,18,23,34] and multivariate [3,10,29,31,42,43] evaluation methods are applied to identify independent prognostic features. However, this large body of predictive data has been partly obscured by relatively small study populations and illdefined series with altering diagnostic criteria.…”

Section: Discussionmentioning

confidence: 99%

Prognostic impact of apoptosis and proliferation in idiopathic (primary) myelofibrosis

Kvasnicka

Thiele

Regn

et al. 1999

Annals of Hematology

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A retrospective study of 120 patients with the clinically and histologically established diagnosis of idiopathic (primary) myelofibrosis (IMF) was performed to determine prognostic factors of predictive value, including parameters characterizing the dynamics of hematopoietic cell kinetics. In contrast to previous studies, our cohort comprised the full spectrum of the disease, from initial prefibrotic to advanced osteosclerotic stages. The in situ end-labeling (ISEL) technique was used to demonstrate apoptosis, in order to determine dynamic parameters of predictive value. Cell proliferation was evaluated by employing the monoclonal antibody PC10 directed against proliferating cell nuclear antigen (PCNA). Proliferative activity (PCNA index) and frequency of apoptosis showed significant differences between early and advanced fibrosclerotic stages of disease. Decrease in proliferation indicated a significantly shorter survival, whereas a higher frequency of apoptotic cells was associated with a better prognosis. It may be speculated that a normal or enhanced proliferation rate expressed by PCNA positivity (late G1- and S-phase of the cell cycle) that is accompanied by a higher incidence of apoptosis reflects the regenerative (turnover) capacity of hematopoiesis. This may apply especially to early hypercellular stages without relevant myelofibrosis. In consideration of a recently published multivariate risk model, a simplified synthesis score for stratification of a patient's prognosis was constructed. Age, degree of anemia, leukocytes, and platelet count were regarded as the most important parameters. A substantial improvement of prognostic efficiency was further achieved by including PCNA index and frequency of apoptosis. Our results are in keeping with the assumption that generalization, indicated by myeloid metaplasia, has a prodigious impact on prognosis in IMF. Furthermore, in this context dynamic features such as proliferative activity and frequency of apoptosis exert an additional predictive value.

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Prognostic factors in myelofibrosis

Cited by 39 publications

References 13 publications

Identification of ‘short‐lived’ and ‘long‐lived’ patients at presentation of idiopathic myelofibrosis

Identification of ‘short‐lived’ and ‘long‐lived’ patients at presentation of idiopathic myelofibrosis

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

Prognostic impact of apoptosis and proliferation in idiopathic (primary) myelofibrosis

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