title: Hemoglobin levels and circulating blasts are two easily evaluable diagnostic parameters highly predictive of leukemic transformation in primary myelofibrosis.
abstract: To predict leukemic transformation (LT), we evaluated easily detectable diagnostic parameters in 338 patients with primary myelofibrosis (PMF) followed in the Latium region (Italy) between 1981 and 2010. Forty patients (11.8%) progressed to leukemia, with a resulting 10-year leukemia-free survival (LFS) rates of 72%. Hb (<10g/dL), and circulating blasts (≥1%) were the only two independent prognostic for LT at the multivariate analysis. Two hundred-fifty patients with both the two parameters available were grouped as follows: low risk (none or one factor)=216 patients; high risk (both factors)=31 patients. The median LFS times were 269 and 45 months for the low and high-risk groups, respectively (P<.0001). The LT predictive power of these two parameters was confirmed in an external series of 270 PMF patients from Tuscany, in whom the median LFS was not reached and 61 months for the low and high risk groups, respectively (P<.0001). These results establish anemia and circulating blasts, two easily and universally available parameters, as strong predictors of LT in PMF and may help to improve prognostic stratification of these patients particularly in countries with low resources where more sophisticated molecular testing is unavailable.
authors: Hematology Unit (2U) ICOT/S, Maria Goretti Hospital, AUSL Latina, Italy.
authors: Angela
authors: A
authors: Rago
authors: Department of Cellular Biotechnology and Hematology, University of Rome "Sapienza", Italy.
authors: Roberto
authors: R
authors: Latagliata
authors: Department of Hematology, Belcolle Hospital, Viterbo, Italy.
authors: Marco
authors: M
authors: Montanaro
authors: Department of Hematology, S. Andrea Hospital, Rome, Italy.
authors: Enrico
authors: E
authors: Montefusco
authors: Department of Hematology, "Nuovo Regina Margherita" Hospital, Rome, Italy.
authors: Alessandro
authors: A
authors: Andriani
authors: Department of Hematology, S. Pertini Hospital, Rome, Italy.
authors: Sabrina Leonetti
authors: SL
authors: Crescenzi
authors: Hematology Unit (2U) ICOT/S, Maria Goretti Hospital, AUSL Latina, Italy.
authors: Sergio
authors: S
authors: Mecarocci
authors: Department of Hematology, S. Camillo Hospital, Rome, Italy.
authors: Francesca
authors: F
authors: Spirito
authors: Department of Hematology, Regina Elena National Cancer Institute, Rome, Italy.
authors: Antonio
authors: A
authors: Spadea
authors: Department of Hematology, S. Spirito Hospital, Rome, Italy.
authors: Umberto
authors: U
authors: Recine
authors: Department of Biomedicine and Prevention, University "Tor Vergata", Rome, Italy.
authors: Laura
authors: L
authors: Cicconi
authors: Hematology, Stem Cell Transplantation, Transfusion Medicine and Cellular Therapy Unit, University "Campus Bio-Medico" of Rome, Italy.
authors: Giuseppe
authors: G
authors: Avvisati
authors: Department of Hematology, S. Giovanni Addolorata Hospital, Rome, Italy.
authors: Michele
authors: M
authors: Cedrone
authors: Department of Cellular Biotechnology and Hematology, University of Rome "Sapienza", Italy.
authors: Massimo
authors: M
authors: Breccia
authors: Department of Hematology, S. Andrea Hospital, Rome, Italy.
authors: Raffaele
authors: R
authors: Porrini
authors: Department of Hematology, "Nuovo Regina Margherita" Hospital, Rome, Italy.
authors: Nicoletta
authors: N
authors: Villivà
authors: Department of Hematology, Belcolle Hospital, Viterbo, Italy.
authors: Cinzia
authors: C
authors: De Gregoris
authors: Department of Cellular Biotechnology and Hematology, University of Rome "Sapienza", Italy.
authors: Giuliana
authors: G
authors: Alimena
authors: Department of Statistic Science, University of Rome "Sapienza", Italy.
authors: Enzo
authors: E
authors: D'Arcangelo
authors: Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
authors: Paola
authors: P
authors: Guglielmelli
authors: Department of Biomedicine and Prevention, University "Tor Vergata", Rome, Italy; Laboratory of Neuro-Oncohematology, Santa Lucia Foundation, Rome, Italy.
authors: Francesco
authors: F
authors: Lo-Coco
authors: Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
authors: Alessandro
authors: A
authors: Vannucchi
authors: Hematology Unit (2U) ICOT/S, Maria Goretti Hospital, AUSL Latina, Italy; Department of Cellular Biotechnology and Hematology, University of Rome "Sapienza", Italy. Electronic address: cimino@bce.uniroma1.it.
authors: Giuseppe
authors: G
authors: Cimino
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000740:Anemia / Q000503:physiopathology*; D001752:Blast Crisis; D002471:Cell Transformation, Neoplastic / Q000378:metabolism / Q000473:pathology*; D005260:Female; D005500:Follow-Up Studies; D006454:Hemoglobins / Q000032:analysis*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009360:Neoplastic Cells, Circulating / Q000473:pathology*; D055728:Primary Myelofibrosis / Q000097:blood / Q000175:diagnosis*; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D054928:Validation Studies as Topic
chemical_list: D006454:Hemoglobins
keywords: Anemia; Circulating blasts; Leukaemic transformation; Leukemic transformation risk; Primary myelofibrosis