ROP Interferon Alpha 2B in Polycythemia Vera

Overview of Polycythemia Vera (PV)

Definition: A myeloproliferative neoplasm characterized by an increase in red blood cells, white blood cells, and platelets.
Risk: Increased risk of thrombosis.
Cause: In 95% of patients, PV begins with an acquired mutation in the JAK2 gene.
- Effect: Drives clonal expansion of mutant hematopoietic stem cells (HSCs).
- Pathway: Mutation leads to constitutive activation of the JAK-STAT pathway, causing overproduction of blood cells.

Function: A cytokine with biological activities that include depleting mutant HSC populations.
Mechanism: Binds to cell surface receptors initiating signaling cascades, affecting gene expression, cellular differentiation, and apoptosis.
Therapeutic Effects: Not fully understood in PV.

Serious Disorders: Interferon Alpha products can cause or exacerbate life-threatening neuropsychiatric, autoimmune, ischemic, and infectious disorders.
Monitoring: Patients should undergo regular clinical and laboratory evaluations.
Discontinuation Criteria: Persistently severe or worsening symptoms merit discontinuation.

Psychiatric Disorders: Severe psychiatric disorders, especially severe depression or suicidal thoughts/history.
Hypersensitivity: To interferons or inactive ingredients of BZ Remy.
Liver Impairment: Moderate or severe hepatic impairment.
Autoimmune Disease: Active or untreated autoimmune diseases.
Transplant History: Receiving immunosuppressants.

Psychiatric: Life-threatening psychiatric reactions possible; monitor closely.
Endocrine: Worsening hypo/hyperthyroidism; discontinue if unmanaged.
Cardiovascular: Monitor in patients with cardiovascular history; avoid in severe cases.
Blood Counts: Monitor for infection or bleeding due to decreased blood counts.
Hypersensitivity: Acute reactions require immediate discontinuation and treatment.
Pancreatitis: Occurs in 2.2% of patients; evaluate if suspected.
Colitis: Discontinue with signs of ulcerative or hemorrhagic colitis.
Pulmonary: Discontinue with pulmonary infiltrates or impairment.
Ophthalmologic: Severe eye disorders monitored with eye exams required.
Lipid Levels: Elevated triglycerides monitored and managed.
Liver Function: Monitor liver enzymes; discontinue if hepatic decompensation occurs.
Renal Function: Monitor creatinine; avoid with severe impairment.
Dental Health: Maintain oral hygiene and regular dental exams.
Dermatologic: Discontinue if significant skin issues occur.
Driving and Machinery: Patients should assess their ability to operate vehicles and machinery.

Common Reactions (>40%): Influenza-like illness, arthralgia, fatigue, pruritis, nasitis, musculoskeletal pain.
Other Reactions (>10%): Liver enzyme elevations, thrombocytopenia, myalgia.

CYP450 Substrates: Monitor patients using CYP450 substrates with narrow therapeutic indices.
Avoidance: With myelosuppressive agents, narcotics, hypnotics, or sedatives.

Pregnancy: Potential for fetal harm; contraception advised for women of reproductive potential.
Lactation: Advise against breastfeeding during treatment and for 8 weeks post-treatment.
Renal Impairment: Avoid if eGFR <30 ml/min.
Pediatric Use: Safety and effectiveness not established.