Belzutifan is the first approved HIF-2 α inhibitor for the treatment of VHL syndrome associated renal cell carcinoma, exerting anti-tumor effects by targeting the hypoxia inducible factor pathway. This guide will provide a detailed introduction to its indications, usage, dosage, and key points for managing adverse reactions.

1. Indications

(1) VHL syndrome associated renal cell carcinoma: suitable for adult patients who do not require immediate surgery.

(2) VHL syndrome related central nervous system hemangioblastoma: can delay tumor growth.

(3) VHL syndrome associated pancreatic neuroendocrine tumors: demonstrating disease control effectiveness.

2. Usage and dosage

(1) Standard dose: Take 120mg orally once a day, whole tablet, and can be taken with food.

(2) Dose adjustment: Suspend administration when grade 3 anemia occurs, and reduce to 80mg to continue treatment after recovery.

(3) Duration of treatment: Continue medication until disease progression or intolerable toxicity occurs.

3. Pharmacological effects

(1) Targeted inhibition of HIF-2 α protein, blocking abnormal angiogenesis caused by VHL gene deficiency.

(2) Specifically targeting the hypoxic signaling pathway, reducing its impact on normal tissues.

4. Adverse reaction management

(1) Anemia: incidence rate of 90%, regular monitoring of hemoglobin, and necessary blood transfusion support.

(2) Hypoxia: Monitor blood oxygen saturation and use with caution for patients with chronic lung disease.

(3) Fatigue: 62% of patients experience it, it is recommended to arrange activities and rest reasonably.

(4) Gastrointestinal reactions: nausea (39%), constipation (31%), can be treated accordingly.

5. Special precautions

(1) Contraceptive requirements: Efficient contraceptive measures should be taken during treatment and one week after discontinuation of medication.

(2) Drug interactions: Avoid using strong CYP3A4 inducers or inhibitors in combination.

(3) Monitoring requirements: Regularly check blood routine, blood oxygen, and liver function.