Polycythemia rubra vera

Polycythemia

vera • Polycythemia - Ganzer Fall bei Radiopaedia

Mousavi, F. Polycythemia. Case study, Radiopaedia.org. (accessed on 20 Oct 2022) https://doi.org/10.53347/rID-53493 • CC by-nc-sa 3.0 (modified)

Polycythemia

vera • Polycythemia vera - Ganzer Fall bei Radiopaedia

Narcise, L. Polycythemia vera. Case study, Radiopaedia.org. (accessed on 20 Oct 2022) https://doi.org/10.53347/rID-87581 • CC by-nc-sa 3.0 (modified)

Knochenmarksveränderungen

bei Polycythaemia vera: In der sagittalen T1-gewichteten MRT der Wirbelsäule zeigt sich rechts bei Polycythaemia vera eine deutliche, diffuse Signalabsenkung des Knochenmarks. Linkes Bild zum Vergleich mit normaler Signalintensität eines ähnlich alten Patienten ohne myeloproliferative Erkrankung.

Wikipedia • CC BY-SA 4.0

Radiopaedia • CC-by-nc-sa 3.0 • de

Polycythemia vera (older term: polycythemia rubra vera) is a myeloproliferative neoplasm that results in an excess of red blood cells in the bloodstream.

Epidemiology

The estimated prevalence is around 2-3 per 10,000 people. It typically presents in older individuals. There may be a slightly greater male predilection.

Pathology

Markers

JAK2 mutations (particularly the V617F mutation) can be found in more than 95% of people with polycythemia vera.

Diagnostic criteria

Major WHO criteria are as follows:

Hemoglobin >16.5 g/dL in men and >16 g/dL in women, or hematocrit >49% in men and >48% in women, or red cell mass >25% above mean normal predicted value

Bone marrow biopsy showing hypercellularity for age with trilineage growth (panmyelosis) including prominent erythroid, granulocytic, and megakaryocytic proliferation with pleomorphic, mature megakaryocytes (differences in size)

Presence of JAK2V617F or JAK2 exon 12 mutation

The minor WHO criterion is as follows:

Serum erythropoietin level below the reference range for normal

Radiographic features

Radiographic manifestations are varied and non-specific and can affect a number of systems, including:

splenomegaly
organ infarcts e.g. splenic infarcts
features of extramedullary hematopoiesis
increased radiographic attenuation of cerebral arteries, veins and dural sinuses (may mimic cerebral vein thrombosis)

Complications

Treatment and prognosis

The overall prognosis can be variable with a wide natural course amongst individuals. Treatment options include venesection, myelosuppressive medications, interferon, and aspirin.

Anagrelide is used when secondary thrombocythemia is an issue. Oral radiophosphorus (32-P) is now avoided in 'benign' myeloproliferative diseases due to the risk of inducing acute myeloid leukemia .