JL418. Primary Polycythemia Vera: Advances in Diagnosis and Treatment
Kathy Leonard, MA, ACNP-B, ANP-B, ANP-BC, AOCNP®, New York University Clinical Cancer Center, New York, NY
Purpose: The purpose of this poster presentation is to educate advanced hematology/oncology practitioners how to diagnose, evaluate thrombotic risk, manage and treat patients with primary polycythemia vera. Background: Primary polycythemia vera is a clonal stem cell disorder characterized by erythrocytosis, as well as leukocytosis and thrombocytosis. It is a Philadelphia chromosome negative subtype of the myeloproliferative neoplasms. The age of diagnosis is usually in the sixth decade and is rarely seen in individuals under the age of thirty. The condition was first described in the late 1800s, and it wasn’t until 2005 that a mutation of the JAK2 (Janus kinase 2) gene was identified as playing a role in this disease. Polycythemia vera may result in life threatening thrombotic events, progression to myelofibrosis and acute myeloid leukemia. The primary treatment is aspirin and therapeutic phlebotomy with platelet lowering in select patient populations. The identification of the JAK 2 V617F mutation has led to a more definitive diagnosis and has made way for the use of JAK inhibitors. Discussion: Treatment of polycythemia vera is aimed at prevention of life threatening thromboembolic events through careful risk stratification, identification and monitoring of side effects of pharmacological agents and early identification of disease progression to myelofibrosis and acute myeloid leukemia. Implications for Advanced Practice: It is very important that advanced hematology/oncology practitioners have a strong knowledge and understanding of the diagnosis, management and treatment of this disease. The advanced practitioner has a pivotal role in treatment and management of those with polycythemia vera.
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