Splenectomy Normalizes Hematocrit in Murine Polycythemia Vera

Splenectomy Normalizes Hematocrit in Murine Polycythemia Vera

Splenic enlargement (splenomegaly) develops in numerous disease states, although a specific pathogenic role for the spleen has rarely been described. In polycythemia vera (PV), an activating mutation in Janus kinase 2 (JAK2(V617)) induces splenomegaly and an increase in hematocrit. Splenectomy is sparingly performed in patients with PV, however, due to surgical complications. Thus, the role of ...

Efficacy of vorinostat in a murine model of polycythemia vera.

The discovery of the JAK2V617F mutation in most patients with Ph-negative myeloproliferative neoplasms has led to the development of JAK2 kinase inhibitors. However, JAK2 inhibitor therapy has shown limited efficacy and dose-limiting hematopoietic toxicities in clinical trials. In the present study, we describe the effects of vorinostat, a small-molecule inhibitor of histone deacetylase, agains...

Muramidase in polycythemia vera.

Erythropoietin receptors in polycythemia vera.

The role of erythropoietin (EP) in polycythemia vera (PV) is controversial, with some experiments suggesting that erythroid progenitors in PV are exquisitely sensitive to EP and EP dependent, and others suggesting that PV progenitors are EP independent. We have examined the characteristics of the EP receptor (EP-R) on erythroid colony-forming cells (ECFC) from patients with PV. In contrast to n...

Treatment of polycythemia vera.

BACKGROUND AND OBJECTIVE Current guidelines for the management of patients with polycythemia vera (PV) derive from a few clinical trials and several uncontrolled clinical studies. The purpose of this paper is to critically review the available evidence in literature for selecting the best treatment in the single patient. METHODS The authors have been working in this field contributing origina...