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Blood, 1 April 2008, Vol. 111, No. 7, pp. 3383-3387.
Prepublished online as a Blood First Edition Paper on January 10, 2008; DOI 10.1182/blood-2007-11-121434.


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CLINICAL TRIALS AND OBSERVATIONS

A dynamic prognostic model to predict survival in post–polycythemia vera myelofibrosis

Francesco Passamonti1, Elisa Rumi1, Marianna Caramella2, Chiara Elena1, Luca Arcaini1, Emanuela Boveri3, Cecilia Del Curto1, Daniela Pietra1, Laura Vanelli1, Paolo Bernasconi1, Cristiana Pascutto1, Mario Cazzola1, Enrica Morra2, and Mario Lazzarino1

1 Department of Hematology, Fondazione Istituto di ricovero e cura a carattere scientifico (IRCCS) Policlinico San Matteo, University of Pavia, Pavia; 2 Department of Hematology, Ospedale Niguarda Ca' Granda, Milan; and 3 Department of Surgical Pathology, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico San Matteo, University of Pavia, Pavia, Italy

Post–polycythemia vera myelofibrosis (post-PV MF) is a late evolution of PV. In 647 patients with PV, we found that leukocytosis leukocyte count > (15 x 109/L) at diagnosis is a risk factor for the evolution of post-PV MF. In a series of 68 patients who developed post-PV MF, median survival was 5.7 years. Hemoglobin level less than 100 g/L (10 g/dL) at diagnosis of post-PV MF was an independent risk factor for survival. The course of post-PV MF, however, is a dynamic process that implies a progressive worsening of clinical parameters. Using a multivariate Cox proportional hazard regression with time-dependent covariates, we found that a dynamic score based on hemoglobin level less than 100 g/L (10 g/dL), platelet count less than 100 x 109/L, and leukocyte count more than 30 x 109/L is useful to predict survival at any time from diagnosis of post-PV MF. The resulting hazard ratio of the score was 4.2 (95% CI: 2.4-7.7; P < .001), meaning a 4.2-fold worsening of survival for each risk factor acquired during follow up. In conclusion, leukocytosis at diagnosis of PV is a risk factor for evolution in post-PV MF. A dynamic score based on hemoglobin level, and platelet and leukocyte count predicts survival at any time from diagnosis of post-PV MF.


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