Background: Splenomegaly (SPML) in PV has been considered a diagnostic hallmark, associated with symptoms, affected clinical trial design, and influenced governmental approval of ropeginterferon α-2b. Whether SPML affects myelofibrosis-free and overall survival (MFS and OS) is unknown. Because of uncertainty regarding these issues, we conducted a retrospective, systematic study evaluating them.
Interpretation: Our unique findings are practice-changing. For the first time, we show that PV pts seldom have SPML at DX, symptomatic or not, but frequently have constitutional symptoms. Spleen size palpable ≥5cm correlates with reduced MFS but not OS. These pts require marrow biopsy to establish degree of fibrosis, which, if present, particularly in younger pts, may prompt disease-modifying therapy. Additionally, PV risk stratification should include SPML among features associated with disease progression »
Critical Illness cover and Polycythemia Vera
How high is your WBC in polycythemia vera?
