Polycythemia vera: Difference between revisions
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==Background== | ==Background== | ||
*Chronic myeloproliferative disorder | [[File:Polycythemia vera, blood smear.jpg|thumb|Blood smear from a patient with polycythemia vera. here are three red blood cell precursors present and slight to moderate anisopoikilocytosis. (Wright-Giemsa stain)]] | ||
*Chronic [[myeloproliferative disorders|myeloproliferative disorder]] | |||
*Abnormal proliferation is seen in all 3 cell lines | *Abnormal proliferation is seen in all 3 cell lines | ||
*Typically in elderly | *Typically in elderly | ||
*Can be asymptomatic or symptomatic | *Can be asymptomatic or symptomatic | ||
*Commonly caused by JAK2 mutation | |||
==Clinical | ==Clinical Features== | ||
[[File:Erythromelalgia.jpg|thumb|Erythromelalgia in a patient with longstanding polycythemia vera. Note reddish limbs and swelling..]] | |||
*Severe burning pain in the hands or feet accompanied by a reddish or bluish coloration of the skin | *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: <ref>http://emedicine.medscape.com/article/205114-differential</ref> | *Suspected with any combination of the following: <ref>http://emedicine.medscape.com/article/205114-differential</ref> | ||
**Abnormally elevated hemoglobin levels (>18 g/dL in men; 16 g/dL in women) | **Abnormally ''elevated'' hemoglobin levels (>18 g/dL in men; 16 g/dL in women) | ||
**Normal oxygen saturation | **Normal oxygen saturation | ||
**Bleeding complications | **Bleeding complications | ||
| Line 14: | Line 17: | ||
**Splenomegaly | **Splenomegaly | ||
**Plethora | **Plethora | ||
**Pruritus after bathing | **[[Pruritus]] after bathing | ||
*May develop [[hyperviscosity syndrome]] | |||
== | ==Differential Diagnosis== | ||
{{Polycythemia DDX}} | |||
==Evaluation== | |||
===Workup=== | |||
*CBC | |||
*Chem 7 | |||
*ESR | |||
*EPO decreased | |||
*Bone marrow biopsy sometimes needed | |||
===Evaluation<ref>Tefferi, A, et al. The 2008 World Health Organization classification system for myeloproliferative neoplasms: order out of chaos. Cancer. 2009; 115(17):3842-7,</ref>=== | |||
*Criteria 1: All category A | *Criteria 1: All category A | ||
*Criteria 2: First 3 of category A and any 2 of category B | *Criteria 2: First 3 of category A and any 2 of category B | ||
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| || Vit B12 >900 | | || Vit B12 >900 | ||
|} | |} | ||
== | ==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> | ||
* | **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-α]]) | ||
*If presenting with [[hyperviscosity syndrome]]: | |||
**[[IVF]] and phlebotomy | |||
== | ==Disposition== | ||
==Also | ==See Also== | ||
*[[Hyperviscosity syndrome]] | |||
==External Links== | |||
== | ==References== | ||
<references/> | <references/> | ||
[[Category:Heme/Onc]] | [[Category:Heme/Onc]] | ||
Latest revision as of 21:46, 11 January 2023
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
- May develop hyperviscosity syndrome
Differential Diagnosis
Polycythemia (Erythrocytosis)
- Primary polycythemia (myeloproliferative disorders)
- Secondary polycythemia (due to increased EPO)
- Chronic hypoxemia (e.g. COPD, physiologic altitude adaptations or chronic mountain sickness)
- Carboxyhemoglobin (chronic smokers)
- Renal cyst or hydronephrosis
- EPO-secreting tumors (RCC, hepatocellular carcinoma)
- Anabolic steroid abuse
- Abnormal hemoglobins
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-α)
- If presenting with hyperviscosity syndrome:
- IVF and phlebotomy
Disposition
See Also
External Links
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.