TY - JOUR
T1 - Leukemic transformation of polycythemia vera
T2 - A single center study of 23 patients
AU - Passamonti, Francesco
AU - Rumi, Elisa
AU - Arcaini, Luca
AU - Castagnola, Carlo
AU - Lunghi, Monia
AU - Bernasconi, Paolo
AU - Della Porta, Matteo Giovanni
AU - Columbo, Nora
AU - Pascutto, Cristiana
AU - Cazzola, Mario
AU - Lazzarino, Mario
PY - 2005/9/1
Y1 - 2005/9/1
N2 - BACKGROUND. Acute leukemia (AL) may occur as rare and late event of polycythemia vera (PV). METHODS. The current study included 23 patients who developed acute leukemia in a cohort of 414 consecutive PV patients with long-term observation (3208 person years of follow-up), Kaplan-Meier Product-Limit method was used to estimate the cumulative probability of survival; Gehan-Wilcoxon test was applied to compare survival in different groups of patients. RESULTS. Median age was 68 years, and 18 patients (78%) were > 60 years of age. At diagnosis of AL, most patients had a white blood count > 10 × 10
9/L (n = 17; 74%), Hgb <10 g/dL (n = 13; 57%), and platelet count > 50 × 10
9/L (n = 17; 74%). Of 14 patients in whom cytogenetic analysis was available at leukemia transformation, 12 showed high-risk abnormalities including complex karyotype (n = 10), del (7)(q22) sole (n = 1) and del (X)(q26) sole (n = 1), whereas 2 had a normal karyotype. In patients whose karyotype was available at diagnosis of PV, cytogenetic evolution was documented at progression to AL. Treatment consisted of supportive care and/or low-dose chemotherapy (n = 15), or induction chemotherapy (n = 8). This included idarubicin plus cytarabine (n = 3), high-dose cytarabine (n = 4), and fludarabine-based regimen (n = 1). Allogenic stem cell transplantation was offered to a single patient, who is alive at Day + 70. The outcome of patients was poor, with a median survival of 2.9 months (range, 0.6-20.1 mos), with no significant differences between palliation and intensive treatments. CONCLUSIONS. AL following PV has distinct clinical and biologic features. Outcome of patients is poor irrespective of the treatment employed.
AB - BACKGROUND. Acute leukemia (AL) may occur as rare and late event of polycythemia vera (PV). METHODS. The current study included 23 patients who developed acute leukemia in a cohort of 414 consecutive PV patients with long-term observation (3208 person years of follow-up), Kaplan-Meier Product-Limit method was used to estimate the cumulative probability of survival; Gehan-Wilcoxon test was applied to compare survival in different groups of patients. RESULTS. Median age was 68 years, and 18 patients (78%) were > 60 years of age. At diagnosis of AL, most patients had a white blood count > 10 × 10
9/L (n = 17; 74%), Hgb <10 g/dL (n = 13; 57%), and platelet count > 50 × 10
9/L (n = 17; 74%). Of 14 patients in whom cytogenetic analysis was available at leukemia transformation, 12 showed high-risk abnormalities including complex karyotype (n = 10), del (7)(q22) sole (n = 1) and del (X)(q26) sole (n = 1), whereas 2 had a normal karyotype. In patients whose karyotype was available at diagnosis of PV, cytogenetic evolution was documented at progression to AL. Treatment consisted of supportive care and/or low-dose chemotherapy (n = 15), or induction chemotherapy (n = 8). This included idarubicin plus cytarabine (n = 3), high-dose cytarabine (n = 4), and fludarabine-based regimen (n = 1). Allogenic stem cell transplantation was offered to a single patient, who is alive at Day + 70. The outcome of patients was poor, with a median survival of 2.9 months (range, 0.6-20.1 mos), with no significant differences between palliation and intensive treatments. CONCLUSIONS. AL following PV has distinct clinical and biologic features. Outcome of patients is poor irrespective of the treatment employed.
KW - Acute leukemia
KW - Chronic myeloproliferative disorders
KW - Essential thrombocythemia
KW - Myelofibrosis
KW - Polycythemia vera
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U2 - 10.1002/cncr.21297
DO - 10.1002/cncr.21297
M3 - Article
C2 - 16047334
AN - SCOPUS:23844483519
VL - 104
SP - 1032
EP - 1036
JO - Cancer
JF - Cancer
SN - 0008-543X
IS - 5
ER -