Splenomegaly, or enlargement of the spleen, is common in patients with Philadelphia-negative myeloproliferative neoplasms, and it is associated with symptoms like early satiety and abdominal pain. The role that splenomegaly has in terms of quality of life and prognosis in primary myelofibrosis is fairly well understood, but it has been less frequently investigated among patients with essential thrombocythemia (ET) and polycythemia vera (PV).
In a recently published study, a group of authors sought to assess the impact that splenomegaly has on thrombotic risk and survival in patients with ET and PV. They studied 238 patients with ET and 165 patients with PV who were treated at a single center between 1997 and 2019.
The median follow-up was 45.96 months (range, 1.5-316.2 months) for patients with ET patients and 58.42 months (range, 1.2-298.39 months) for patients with PV. The investigators found that the frequency of thrombosis and cardiovascular events was higher in patients with splenomegaly than in patients without splenomegaly at the time of diagnosis in both groups of patients:
- Thrombosis and cardiovascular events occurred in 39.87% of patients with ET and with splenomegaly versus 24.37% of patients without splenomegaly (P = .04).
- In patients with PV, thrombosis or cardiovascular events occurred in 44.44% of patients with splenomegaly versus in 30.39% of patients without splenomegaly (P = .02).
The authors note that increased spleen size has not been shown to be a significant prognostic factor in major models, but, “In accordance with our data and our experience, we believe that the prognostic value of splenomegaly is underestimated in ET and PV and that should be evaluated the possibility to include it as an item of a prognostic scoring system.”
According to the authors, more studies will be needed to evaluate the role that splenomegaly plays in overall survival and thrombotic risk for patients with ET and PV.
Accurso V, Santoro M, Raso S, et al. Splenomegaly impacts prognosis in essential thrombocythemia and polycythemia vera: a single center study. Hematol Rep. 2019;11(4):8281. doi: 10.4081/hr.2019.8281.