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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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Submitted November 5, 2007
Accepted January 3, 2008
A dynamic prognostic model to predict survival in post-polycythemia vera myelofibrosis
Francesco Passamonti*, Elisa Rumi, Marianna Caramella, Chiara Elena, Luca Arcaini, Emanuela Boveri, Cecilia Del Curto, Daniela Pietra, Laura Vanelli, Paolo Bernasconi, Cristiana Pascutto, Mario Cazzola, Enrica Morra, and Mario Lazzarino
Department of Hematology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy
Department of Hematology, Ospedale Niguarda Ca Granda, Milano, Italy
Department of Surgical Pathology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy
* Corresponding author; email: f.passamonti{at}smatteo.pv.it.
Post-polycythemia vera myelofibrosis (post-PV MF) is a late evolution of PV. Within 647 patients with PV we found that leukocytosis (white blood cell count >15 x109/L) at diagnosis is a risk factor for the evolution in post-PV MF. In a series of 68 patients who developed post-PV MF median survival was 5.7 years. Hemoglobin level <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 <10 g/dL, platelet count <100 x109/L, and white blood cell count >30 x109/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, platelet and white blood cell count predicts survival at any time from diagnosis of post-PV MF.

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