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Polycythemia Vera

Imola K. Osapay, MD and Scott D. Everett, MD Reviewed 06/2018
 


BASICS

DESCRIPTION

  • Polycythemia vera (PV) is a chronic myeloproliferative clonal stem cell disorder marked by increased production of red blood cells (erythrocytosis) with excessive erythroid, myeloid,...

DIAGNOSIS

HISTORY

  • Patients may be asymptomatic or present with nonspecific complaints, including fatigue, malaise, weight loss, sweating, and subjective weakness.

  • Erythromelalgia (burning pain of feet/h...

TREATMENT

GENERAL MEASURES

  • Risk factors: Patients >60 years with history of thrombosis are high risk. Those who are <60 years with no history of thrombosis but with elevated platelets (>150,00...

ONGOING CARE

FOLLOW UP RECOMMENDATIONS

Patient Monitoring

Monitor hematocrit often and phlebotomize as needed to maintain target goal. 

DIET

  • Avoid high-sodium diet; can cause fluid retention

  • Avoid iron sup...

REFERENCES

1
Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood.  2016;127(20):2391–2405. [Vi...

ADDITIONAL READING

  • Passamonti F. How I treat polycythemia vera. Blood.  2012;120(2):275–284. [View Abstract on OvidMedline]

  • Tefferi A, Fonseca R. Selective serotonin reuptake inhibitors are effec...

SEE ALSO

Myeloproliferative Neoplasms 

CODES

ICD10

  • D45 Polycythemia vera

  • D75.1 Secondary polycythemia

ICD9

  • 238.4 Polycythemia vera

  • 289.0 Polycythemia, secondary

SNOMED

  • 109992005 Polycythemia vera (disorder)

  • 127062003 Erythrocytosis (disorder)

CLINICAL PEARLS

  • Erythrocytosis: Hgb >16.5 g/dL in men, >16.0 g/dL in women

  • JAK2 mutations are an important component of myeloproliferative disorders.

  • Bone marrow biopsy can help in diagnosis showi...

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