What is the drug of choice for polycythemia?

Hydroxyurea: The drug of choice for polycythemia vera and essential thrombocythemia.

How does JAK2 mutation cause polycythemia vera?

The V617F somatic mutation in the Janus kinase (JAK) 2 gene (JAK2), which causes the substitution of phenylalanine for valine at position 617, has recently been found in the majority of patients with polycythemia vera and in many with essential thrombocythemia or idiopathic myelofibrosis.

How do JAK2 inhibitors work?

Janus kinase inhibitors, also known as JAK inhibitors or jakinibs, are a type of medication that functions by inhibiting the activity of one or more of the Janus kinase family of enzymes (JAK1, JAK2, JAK3, TYK2), thereby interfering with the JAK-STAT signaling pathway.

What is the treatment for JAK2 mutation?

Ruxolitinib (Jafaki®) is a drug that targets JAK2 and other associated mutations. It can reduce the size of the spleen and lessen many myelofibrosis symptoms. People with myelofibrosis often have anemia. This can be treated with blood transfusions.

What is the role of JAK2 in polycythemia vera?

Polycythemia vera is a myeloproliferative neoplasm characterized by clonal hematopoiesis and an absolute increase in the red blood cell mass, with an associated leukocytosis and thrombocytosis. Virtually all patients with PV harbor a mutation in the nonreceptor tyrosine kinase JAK2, with the majority of patients harboring the classic JAK2.

Are there any inhibitors of JAK2 that work?

Several other inhibitors with activity against JAK2 are under investigation. Pacritinib (SB1518) is a JAK2 inhibitor with activity against the wild-type kinase as well as the JAKV617F-mutated kinase; it also inhibits fms-like tyrosine kinase 3 (FLT3). It achieves high rates of reduction in spleen volume. The tested dose is 400 mg daily.

Which is the best JAK2 inhibitor for myeloproliferative neoplasms?

BH3 mimetics promote mitochondrial apoptosis, and synergism with ruxolitinib in MPN cells and animal models has been shown. Synergism between ruxolitinib and the selective inhibitor of nuclear export selinexor has also been demonstrated preclinically.

Are there any JAK inhibitors for psoriatic arthritis?

Although there is overlap, each JAK inhibitor has unique cytokine targets. Historically JAK inhibitors have been used in conditions such as rheumatoid arthritis, psoriatic arthritis, myelofibrosis or polycythemia vera. JAK inhibitors have promising therapeutic potential in COVID-19 due to both their anti-inflammatory and anti-viral effects.