Polycythemia vera

Page most recently updated 7 September 2026

This page focuses on the reported psychiatric associations, which may be the initial presenting manifestation, and on other clinical features that may assist the psychiatrist or primary care physician in identifying the underlying diagnosis, considered through the lenses of the diagnostic lenses framework.

Polycythemia vera is a chronic myeloproliferative condition characterized by clonal proliferation of erythroid cells in the bone marrow, resulting in increased red blood cell mass and often accompanied by leukocytosis and thrombocytosis.

It can present with psychiatric features such as depression, anxiety, cognitive difficulties, and, rarely, psychotic symptoms. Neuropsychiatric manifestations may occur in association with hyperviscosity and microvascular disturbances, with reported neurological features including headache, dizziness, visual disturbances, transient ischemic symptoms, and, rarely, movement disorders such as chorea.

Other characteristic features include aquagenic pruritus, particularly after a warm bath or shower; erythromelalgia, characterized by burning pain, warmth, and erythema of the hands and feet; facial plethora; headache and light-headedness; visual disturbances; and splenomegaly. Thrombotic events may be the presenting manifestation and can involve arterial or venous circulation, including unusual sites such as the hepatic or portal veins.

From a multisystemic lens perspective, clinical features in other body systems include (but are not limited to):

  • Cardiovascular: Arterial and venous thrombosis, including myocardial infarction, ischemic stroke, deep-vein thrombosis, pulmonary embolism, and other macrovascular thrombotic events; microvascular disturbances may cause erythromelalgia and transient ocular or cerebral ischemic symptoms.

  • Hepatobiliary: Hepatic or portal vein thrombosis, including Budd–Chiari syndrome (hepatic venous outflow obstruction), which may be an initial manifestation of polycythemia vera. Hepatomegaly may occur with hepatic involvement or extramedullary haematopoiesis.

  • Gastrointestinal: Gastrointestinal bleeding and abdominal fullness or discomfort related to splenomegaly.

  • Hematologic: Erythrocytosis is the defining feature, often accompanied by leukocytosis and thrombocytosis. Thrombotic and bleeding complications may occur; bleeding is particularly associated with marked thrombocytosis or acquired von Willebrand syndrome. Progressive disease can evolve into post-polycythemia vera myelofibrosis or, less commonly, acute myeloid leukemia.

  • Dermatologic: Aquagenic pruritus and erythromelalgia are particularly characteristic. Facial plethora and generalized erythema may also occur.

Early diagnosis is important to reduce the risk of potentially life-threatening thrombotic and bleeding complications, identify patients who require risk-adapted treatment, monitor for progression to myelofibrosis or acute myeloid leukemia, and improve long-term prognosis and quality of life.

Selected references and further reading — Multisystemic lens

Cardinal RN, Bullmore ET. The diagnosis of psychosis. Cambridge: Cambridge University Press; 2011.

Sachdev PS, Keshavan MS, editors. Secondary schizophrenia. Cambridge: Cambridge University Press; 2010.

Levenson JL, editor. The American Psychiatric Association Publishing textbook of psychosomatic medicine and consultation-liaison psychiatry. 3rd ed. Washington (DC): American Psychiatric Association Publishing; 2019.

Stern TA, Beach SR, Smith FA, Freudenreich O, Vranceau AM, Fava M, editors. Massachusetts General Hospital handbook of general hospital psychiatry. 8th ed. Philadelphia: Elsevier; 2025.

Arciniegas DB, Yudofsky SC, Hales RE, editors. The American Psychiatric Association Publishing textbook of neuropsychiatry and clinical neurosciences. 6th ed. Washington (DC): American Psychiatric Association Publishing; 2018.

Agrawal N, Faruqui R, Bodani M, editors. Oxford textbook of neuropsychiatry. Oxford: Oxford University Press; 2020.

Boland R, Verduin M, editors. Kaplan and Sadock's comprehensive textbook of psychiatry. 11th ed. Philadelphia: Wolters Kluwer; 2024.

Loscalzo J, Fauci AS, Kasper DL, Hauser SL, Longo DL, Jameson JL, editors. Harrison's principles of internal medicine. 22nd ed. New York: McGraw Hill; 2025.