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Polycythemia Vera

Alexander Sasha Rackman, M.D. and Lislie Lisete Gonzalez Veitia, MD Reviewed 04/2024
 


BASICS

DESCRIPTION

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

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

Monitor for secondary malignancies such myeloproliferative neoplasms 

Patient Monitoring

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

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.  2...

ADDITIONAL READING

Kim TY, Kwag D, Lee JH, et al. Clinical Features, Gene Alterations, and Outcomes in Prefibrotic and Overt Primary and Secondary Myelofibrotic Patients. Cancers ...

SEE ALSO

Myeloproliferative Neoplasms 

CODES

ICD10

  • D45 Polycythemia vera

SNOMED

  • 109992005 Polycythemia vera (clinical)

  • 127066000 Familial polycythemia vera

  • 44865000 Secondary polycythemia

  • 389147003 Relative polycythemia

  • 127062003 Erythrocytosis

  • 36...

CLINICAL PEARLS

  • Erythrocytosis: Hgb >16.5 g/dL in men, >16 g/dL in women

  • JAK2 mutations are an important component of myeloproliferative disorders.

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

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