Polycythemia vera: Difference between revisions
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==Management== | ==Management== | ||
*Phlebotomy with goal hematocrit < 45%<ref>Marchioli R et al. Cardiovascular events and intensity of treatment in polycythemia vera. N Engl J Med. 2013; 368(1):22-33.</ref> | *Phlebotomy with goal hematocrit < 45%<ref>Marchioli R et al. Cardiovascular events and intensity of treatment in polycythemia vera. N Engl J Med. 2013; 368(1):22-33.</ref> | ||
**Patients with severe plethora, with [[AMS]] or vascular compromise can be bled vigorously with removal of 500 mL of whole blood | **Patients with severe plethora, with [[AMS]] or vascular compromise can be bled vigorously with removal of 500 mL of whole blood rapidly | ||
**Otherwise, gradual phlebotomy of 500-1000 mL over 24 hours is preferred in less emergent cases | |||
*ASA 81mg | *ASA 81mg | ||
*Myelosuppressive agents (hydroxyurea, interferon alpha) | *Myelosuppressive agents (hydroxyurea, interferon alpha) | ||
Revision as of 21:53, 24 October 2018
Background
- Chronic myeloproliferative disorder
- Abnormal proliferation is seen in all 3 cell lines
- Typically in elderly
- Can be asymptomatic or symptomatic
- Commonly caused by JAK2 mutation
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 with goal hematocrit < 45%[3]
- Patients with severe plethora, with AMS or vascular compromise can be bled vigorously with removal of 500 mL of whole blood rapidly
- Otherwise, gradual phlebotomy of 500-1000 mL over 24 hours is preferred in less emergent cases
- 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,
- ↑ Marchioli R et al. Cardiovascular events and intensity of treatment in polycythemia vera. N Engl J Med. 2013; 368(1):22-33.