Details

Autor(en) / Beteiligte

• PASSAMONTI, Francesco
• RUMI, Elisa
• LAZZARINO, Mario
• ARCAINI, Luca
• CASTAGNOLA, Carlo
• LUNGHI, Monia
• BERNASCONI, Paolo
• PORTA, Matteo Giovanni
• COLUMBO, Nora
• PASCUTTO, Cristiana
• CAZZOLA, Mario

Titel

Leukemic transformation of polycythemia vera : A single center study of 23 patients

Ist Teil von

• Cancer, 2005-09, Vol.104 (5), p.1032-1036

Ort / Verlag

New York, NY: Wiley-Liss

Erscheinungsjahr

2005

Link zum Volltext

Quelle

Wiley Online Library - AutoHoldings Journals

Beschreibungen/Notizen

• Acute leukemia (AL) may occur as rare and late event of polycythemia vera (PV). 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. 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 x 10(9)/L (n = 17; 74%), Hgb < 10 g/dL (n = 13; 57%), and platelet count > 50 x 10(9)/L (n = 17; 74%). Of 14 patients in whom cytogenetic analysis was available at leukemic 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. AL following PV has distinct clinical and biologic features. Outcome of patients is poor irrespective of the treatment employed.