Myeloproliferative neoplasms (MPN)

The most clinically relevant MPN include chronic myeloid leukemia (CML), polycythemia vera (PV), primary myelofibrosis (PMF), and essential thrombocythemia (ET).

Chronic eosinophilic leukemia is also a type of MPN.

All myeloproliferative neoplasms may lead to elevated uric acid levels and gout as a result of increased cellular breakdown. They are also associated with an increased risk of acute myeloid leukemia.

Chronic Myelogenous Leukemia (CML)

Essential Thrombocytosis/Thrombocythemia

most common MPN

char. by thrombotic and hemorrhagic complications

a stem cell disorder marked by a predominant increase in megakaryocytes and platelet count >600,000, in the absence of secondary thrombocytosis

S/S

vasomotor disturbances such as erythromelalgia

livedo reticularis

headache

vision symptoms

arterial or venous thromboses

Splenomegaly (up to 50%)

Dx

JAK2 mutation in half of the cases; A negative JAK2 does not exclude the Dx

Tx