Tiziano BARBUI
FROM Research Foundation, Ospedale Papa Giovanni XXIII
Title: Polycythemia Vera: Clinical Evidence to use Interferon-alfa in the real world clinical practice
Biography
Biography: Tiziano BARBUI
Abstract
Polycythemia vera (PV) is a myeloproliferative neoplasm (MPN) characterized by uncontrolled clonal proliferation of multipotent bone marrow progenitors, sustained by acquired genetic mutations in JAK2 genes (JAK2 V617F and exon 12 mutations). Expansion of the mutated clone triggers an inflammatory response leading to vascular complications and disease progression into myelofibrosis (MF) and acute leukemia (AL). For each single outcome (thrombosis, bleeding, evolution into MF or AL, symptoms and quality of life, pregnancy) a specific review of the literature to grade the quality of direct and indirect evidence supporting a net benefit of cytoreductive therapy start, or change, in each subgroup was examined and recommendations were assigned to subgroups of achieving a consensus >=85% or supported by a high-quality evidence for at least one outcome or a moderate evidence for a critical outcome. Weak recommendations will be labelled by a “to be considered” wording. For clinical question concerning the indication and choice of single drugs, all reviewed data will be translated into Patient-Intervention-Comparator-Outcome questions (PICO) and clinical recommendations were produced by a GRADE process. In those question without a sufficient evidence, a consensus will be required. During the presentation the results of the POINT-C project will be reported and specific recommendation will be discussed. The major problem of PV patients is represented by major arterial and venous thrombosis vascular complications whose rate is the highest at the time of diagnosis and shortly thereafter (ref x,xx). In the real world clinical practice of contemporary 1500 patients with PV, total major thrombosis rate was 2.62% patients per year, an estimate lower than that reported in the ECLAP trial (4.4% patients per year) but comparable to the rate seen in the recent Cyto-PV study (2.7% patients per year, venous and arterial thrombosis in 1.59% and 1.05% patients per year respectively.