Originally published as JCO Early Release 10.1200/JCO.2009.22.8833 on September 28 2009

This Article Full Text Full Text (PDF) Purchase Article View Shopping Cart Alert me when this article is cited Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Save to my personal folders Download to citation manager Rights & Permissions Google Scholar Articles by Tam, C. S. Articles by Verstovsek, S. PubMed PubMed Citation Articles by Tam, C. S. Articles by Verstovsek, S. Social Bookmarking                            What's this?

Dynamic Model for Predicting Death Within 12 Months in Patients With Primary or Post–Polycythemia Vera/Essential Thrombocythemia Myelofibrosis

From the Leukemia Department, The University of Texas M. D. Anderson Cancer Center, Houston, TX; and Hematology Department, St Vincent's Hospital, Melbourne, Victoria, Australia.

Corresponding author: Srdan Verstovsek, MD, PhD, Leukemia Department, The University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX 77030; e-mail: sverstov{at}mdanderson.org.

Purpose Current prognostic tools in myelofibrosis (MF) fail to identify patients at the highest risk of death and are limited by their applicability only to the time of diagnosis. We aimed to define an accelerated phase (AP) in MF by characterizing disease features that can identify patients with median overall survival of 12 months at any time in the disease course.

Patients and Methods Baseline characteristics of 370 consecutive patients with MF from a single center were analyzed to identify features associated with a median overall survival of 12 months. These putative AP features were then validated by following the course of chronic-phase patients (no AP features at baseline) until the development of one or more AP features and determining their subsequent survival.

Results The following three characteristics were associated with poor survival at baseline and were selected as putative AP features: blasts in blood or bone marrow 10%, platelets less than 50 x 10⁹/L, and chromosome 17 aberrations (median overall survival, 10, 12, and 5 months, respectively). In the validation phase, chronic-phase patients who developed AP features during follow-up were found to have short subsequent survival times (median overall survival, 12, 15, and 6 months, respectively). AP was a necessary step in the progression to blast phase, with leukemic transformation being exceedingly rare (3% risk at 10 years) in patients who remained persistently in chronic phase.

Conclusion Blood or bone marrow blasts 10%, platelets less than 50 x 10⁹/L, and chromosome 17 aberrations defined AP in patients with MF. Patients in AP should be candidates for intensive therapeutic interventions.

Presented in part at the 49th Annual Meeting of the American Society of Hematology, December 8-11, 2007, Atlanta, GA.

Authors' disclosures of potential conflicts of interest and author contributions are found at the end of this article.

CiteULike    Complore    Connotea    Del.icio.us    Digg    Facebook    Reddit    Technorati    Twitter    What's this?