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Blood, 1 February 2005, Vol. 105, No. 3, pp. 973-977.
Prepublished online as a Blood First Edition Paper on September 23, 2004; DOI 10.1182/blood-2004-07-2864.
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Submitted July 30, 2004
Accepted September 16, 2004
Leukemic transformation in myelofibrosis with myeloid metaplasia: a single institution experience with 91 cases
Ruben A Mesa*, Chin-Yang Li, Rhett P Ketterling, Georgene S Schroeder, Ryan A Knudson, and Ayalew Tefferi
Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, MN, USA
Division of Hematopathology, Mayo Clinic, Rochester, MN, USA
Division of Laboratory Genetics, Mayo Clinic, Rochester, MN, USA
Cancer Center Statistics Unit, Mayo Clinic, Rochester, MN, USA
* Corresponding author; email: mesa.ruben{at}mayo.edu.
Among 2333 consecutive patients with myelofibrosis with myeloid metaplasia (MMM) seen at our institution, 91 fulfilled the WHO criteria for leukemic transformation (LT). All episodes of LT were myeloid in origin (AML) with all FAB subtypes represented except M3; most frequent were M7 (25.4%), M0 (22.4%), and M2 (17.9%). Cytogenetic studies during LT were available in 51 patients and revealed a clonal abnormality in 49 (91%); 30 patients had complex karyotype, 2 had core binding factor gene lesions, and 18 had abnormalities of chromosome 5 or 7. Karyotypic evolution was documented in the majority of the patients in whom serial analysis was possible. In general, LT was fatal in 98% of the cases after a median of 2.6 months (range, 0-24.2). Twenty-four patients received AML-like induction chemotherapy that resulted in no complete remission, 41% reversion into chronic phase disease, and 33% treatment-related mortality. The remaining 67 patients received either supportive care alone (48 patients) or low-intensity chemotherapy (19 patients). Overall, survival was similarly poor in all 3 treatment categories. The outcome of LT in MMM with current therapies is dismal and either supportive care alone or appropriate clinical trials should be considered.
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