Case Presentation: Patient With Polycythemia Vera
Patient Case:
Firas El Chaer, MD, UVA Cancer Center, Charlottesvillle, Virginia, provides background on a patient with polycythemia vera. In this case, the patient is a 29-year-old female with past medical issues of endometriosis and anxiety.
She originally presented with erythromelagia, bone pain, joint pain, fatigue, and right lower extremity swelling. Upon further investigation, her labs showed a hemoglobin of 18 grams per deciliter. Her white panel was actually normal with a normal differential, and her platelet count were normal at 357,000.
Further testing showed that her erythropoietin level was 1.2 mlU/mL, which was low or suppressed. She subsequently underwent a bone marrow biopsy, which revealed hypercellular marrow with sprouting erythropoiesis. There was no increase in blasts. However, there were increased atypical megacaryocytes. The reticulin stain showed no increase in fibrosis. Chromosomal analysis was normal and next [generation] sequencing showed a JAK2 V617F mutation, which was detected at the variant elite frequency of 46%. Because of that constellation of symptoms and diagnostic workup, the patient was diagnosed with polycythemia vera.
In her right lower extremity, subsequent ultrasound showed a deep vein thrombosis. She was started on anticoagulation and the referring provider started her on hydroxyurea as a form of cytoreduction. A few months later, the patient started developing recurrent unexplained fevers. She had an extensive infectious workup, which was negative.
Hydroxyurea was thought to be the culprit. It was stopped and the fever resolved. However, the referring oncologist restarted hydroxyurea and fever reoccurred. The patient was counseled to switch therapy and interferon therapy was offered, but the patient was concerned about the side effects.
The patient was referred to Dr El Chaer's clinic for further evaluation. At this point her counts were controlled and within normal limits. Her hemoglobin was 12 g/dL, her white blood cell count was normal at 5.6K/mcL and her platelets were normal at 312k/mcL.
Take the quiz and watch the video below to follow along with Dr El Chaer's treatment decisions for this patient case.
