From the Guidelines
The statement that is false regarding Polycythemia Vera is (c) increased EPO.
Key Points
- Polycythemia Vera (PV) is characterized by an increased red blood cell mass, which is a key diagnostic criterion 1.
- The JAK2 mutation is a major criterion for the diagnosis of PV, and it is present in approximately 95% of cases 1.
- Serum EPO levels are typically low in PV, even in the presence of treatment with phlebotomy 1.
- Pruritus with warm water and reduced ESR are common symptoms and findings in PV, but they are not specific diagnostic criteria.
- Erythrocytosis is a key feature of PV, and it is often associated with an increased hemoglobin level and hematocrit value 1.
Diagnostic Criteria
The diagnosis of PV requires the presence of two major criteria:
- Laboratory evidence of increased hemoglobin, hematocrit, or red cell mass
- The presence of a JAK2 mutation Additionally, at least one minor criterion is required, which can include MPD-consistent bone marrow histology, serum erythropoietin level below the reference range for normal, or presence of endogenous erythroid colonies 1.
Clinical Implications
The diagnosis of PV is important because it can help guide treatment and management decisions. Phlebotomy is often used to reduce the risk of thrombotic events, and other treatments such as aspirin and cytoreductive therapy may also be used 1.
From the Research
Characteristics of Polycythemia Vera
- JAK2 mutation: The JAK2 V617F mutation is a common feature of Polycythemia Vera (PV), found in over 95% of patients 2.
- Increased red blood cell mass: Erythrocytosis, or an increase in red blood cell mass, is a hallmark of PV 2, 3.
- Pruritus with warm water: Pruritus is a common symptom of PV, affecting approximately 33% of patients, and can be triggered by warm water 2.
- Reduced ESR: The erythrocyte sedimentation rate (ESR) is often reduced in PV due to the increased red blood cell mass 3.
- Erythrocytosis: Erythrocytosis is a required diagnostic criterion for PV, characterized by a hemoglobin level greater than 16.5 mg/dL in men or 16.0 mg/dL in women 2.
False Statement
- Increased EPO: While EPO levels are often reduced in PV, elevated EPO levels can occur in certain cases, such as in patients with Budd-Chiari syndrome 4. However, increased EPO is not a characteristic feature of PV, and reduced EPO levels are more commonly associated with the disease. Therefore, statement (c) increased EPO is the false statement regarding Polycythemia Vera.