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Polycythemia vera
From WikEM
Contents
Background
- Chronic myeloproliferative disorder
- Abnormal proliferation is seen in all 3 cell lines
- Typically in elderly
- Can be asymptomatic or symptomatic
Clinical Features
- Severe burning pain in the hands or feet accompanied by a reddish or bluish coloration of the skin
- Suspected with any combination of the following: [1]
- Abnormally elevated hemoglobin levels (>18 g/dL in men; 16 g/dL in women)
- Normal oxygen saturation
- Bleeding complications
- Portal vein thrombosis
- Splenomegaly
- Plethora
- Pruritus after bathing
Differential Diagnosis
- Essential thrombocytosis (ET)
- Chronic myelogenous leukemia (CML)
- Leukemoid reactions
- Hypoxia
Evaluation
Workup
- CBC
- Chem 7
- ESR
- EPO decreased
- Bone marrow biopsy sometimes needed
Evaluation[2]
- Criteria 1: All category A
- Criteria 2: First 3 of category A and any 2 of category B
| Category A | Category B |
|---|---|
| Increased RBC Mass (Men >18.5, Women >16.5) | Thrombocytosis (>400,000) |
| Normal SPO2 (>92%) | Leukocytosis (>12,000) |
| Splenomegaly | Leukocyte Alk Phos >100 |
| Vit B12 >900 |
Management
- Phlebotomy
- ASA 81mg
- Myelosuppressive agents (hydroxyurea, interferon alpha)
Also See
References
- ↑ http://emedicine.medscape.com/article/205114-differential
- ↑ Tefferi, A, et al. The 2008 World Health Organization classification system for myeloproliferative neoplasms: order out of chaos. Cancer. 2009; 115(17):3842-7,
Authors
Aaron Snyder, Neil Young, Ross Donaldson, Claire, Kevin Lu, Catie Reynolds
