Polycythemia vera: Difference between revisions

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==Background==
==Background==
*Chronic myeloproliferative disorder
*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 Features==
==Clinical Features==
*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
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**Plethora
**Plethora
**[[Pruritus]] after bathing
**[[Pruritus]] after bathing
*May develop [[hyperviscosity syndrome]]


==Differential Diagnosis==
==Differential Diagnosis==
*Essential thrombocytosis (ET)
*Primary polycythemia ([[myeloproliferative disorders]])
*[[Chronic myelogenous leukemia]] (CML)
**[[Polycythemia vera]]
*Leukemoid reactions
**[[Essential thrombocytosis]] (ET)
*[[Hypoxia]]
**[[Chronic myelogenous leukemia]] (CML)
**[[Leukemoid Reaction]]
**[[Myelofibrosis]]
*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==
==Evaluation==
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==Management==
==Management==
*Phlebotomy  
*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>
*ASA 81mg
**Patients with severe plethora, with [[AMS]] or vascular compromise can be bled vigorously with removal of 500 mL of whole blood rapidly
*Myelosuppressive agents (hydroxyurea, interferon alpha)
**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 plebotomy


==Also See==
==See Also==
*[[Hyperviscosity syndrome]]


==References==
==References==

Revision as of 15:08, 12 February 2021

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

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 plebotomy

See Also

References

  1. http://emedicine.medscape.com/article/205114-differential
  2. Tefferi, A, et al. The 2008 World Health Organization classification system for myeloproliferative neoplasms: order out of chaos. Cancer. 2009; 115(17):3842-7,
  3. Marchioli R et al. Cardiovascular events and intensity of treatment in polycythemia vera. N Engl J Med. 2013; 368(1):22-33.