Podcast
Questions and Answers
Which of the following describes the primary mutation found in most patients with Polycythemia Vera?
Which of the following describes the primary mutation found in most patients with Polycythemia Vera?
What is the hallmark clinical manifestation of Polycythemia Vera?
What is the hallmark clinical manifestation of Polycythemia Vera?
What is the recommended frequency for therapeutic phlebotomy in patients with Polycythemia Vera to maintain a hematocrit at 28 mm/h?
What is the recommended frequency for therapeutic phlebotomy in patients with Polycythemia Vera to maintain a hematocrit at 28 mm/h?
Which of the following statements about ESR is true?
Which of the following statements about ESR is true?
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In what scenario would an ESR value exceed 90 mm/h?
In what scenario would an ESR value exceed 90 mm/h?
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Study Notes
Polycythemia Vera (PV)
- PV is a panmyelosis, excessive proliferation of RBCs, megakaryocytes, and granulocytes
- Manifests with erythrocytosis, leukocytosis, and thrombocytosis
- Most patients (90%-97%) have a JAK2V617 mutation
- Treatment of choice is therapeutic phlebotomy to maintain hematocrit below 45%
Erythrocyte Sedimentation Rate (ESR)
- ESR measures the rate of RBCs settling to the bottom of a tube
- Used to detect and monitor inflammatory conditions, like rheumatoid arthritis
- Ordered with other tests to diagnose and monitor inflammatory conditions
- Low ESR values may occur in sickle cell disease (SCD) during a painless crisis, while ESR increases after pain starts.
- ESR increases within one week of SCD crisis
- High ESRs (>28 mm/hr) associated with poorer prognosis in stroke patients
- ESR is greater than or equal to 37 mm/hr associated with a higher risk of prostate cancer progressing
- High ESR (>22 mm/hr in white men) linked to higher risk of coronary artery disease (CAD)
- ESR values greater than or equal to 100 mm/hr in patients with cancer frequently indicates metastases.
- High ESRs are common in active inflammatory diseases (e.g., rheumatoid arthritis, infections, collagen diseases).
- ESR has low specificity and sensitivity, not suitable for screening; prone to technical errors
ESR in Specific Conditions
- Sickle cell disease (SCD): Low ESR during painless crises; increases after crisis onset.
- Osteomyelitis: Elevated ESR
- Stroke: ESR of >28 mm/h, poorer prognosis
- Prostate cancer: ESR ≥ 37 mm/h, higher incidence of disease progression and death
- Coronary artery disease: Elevated ESR associated with a CAD risk(s) in white men
- Cancer: High ESR (> 100 mm/hr), often associated with the presence of metastases
- **Polymyalgia rheumatica/Temporal arteritis:**High ESR (typically exceeding 90mm/hr)
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Description
This quiz covers key concepts related to Polycythemia Vera (PV) and Erythrocyte Sedimentation Rate (ESR). Learn about the features, diagnosis, treatment, and implications of these hematological conditions. Test your knowledge on important mutations and their clinical significance.
