Contents

SynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferences

View all Images (2)

Polycythemia vera
Other Resources UpToDate PubMed

Polycythemia vera

Contributors: Nina Haghi MD, Paritosh Prasad MD
Other Resources UpToDate PubMed

Synopsis

Polycythemia vera (PV) is a rare chronic myeloproliferative disorder in which the bone marrow is characterized by clonal myeloproliferation causing erythrocytosis, increased red blood cell mass, and elevated hemoglobin and hematocrit. PV is associated with an increased risk of thrombotic events and bone marrow fibrosis, and can transform into acute leukemia. It can occur in patients of any age, with a slight male predominance. Median age at diagnosis is 60 years. The condition can begin in childhood and has been associated with mutations in the erythropoietin receptor gene. Rarely, Pacak-Zhuang syndrome may be seen in patients with early-onset polycythemia and paragangliomas.

The peripheral blood smear often demonstrates normochromic, normocytic red blood cells (may be hypochromic and microcytic if iron deficiency is also present), thrombocytosis, and leukocytosis.

The bone marrow may show hypercellularity, megakaryocytic proliferation, fibrosis, and the absence of stainable iron. If the marrow shows fibrosis, the spleen will enlarge due to extramedullary hematopoiesis. More than 95% of cases of PV have a JAK2 mutation (which can be detected on peripheral blood or bone marrow).

PV is a chronic condition with many phases. When the disease is more progressed, there is possibility of increased fibrosis or that it may transform into acute leukemia.

Codes

ICD10CM:
D45 – Polycythemia vera

SNOMEDCT:
128841001 – Polycythemia vera

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

To perform a comparison, select diagnoses from the classic differential

Subscription Required

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Reviewed:12/17/2017
Last Updated:01/20/2022
Copyright © 2024 VisualDx®. All rights reserved.
Polycythemia vera
A medical illustration showing key findings of Polycythemia vera : Blurred vision, Dizziness, Headache, Splenomegaly, PLT increased, RBC increased
Copyright © 2024 VisualDx®. All rights reserved.