Desirox

Deferasirox (Deferasirox) instructions
Common name: Deferasirox
Trade name: Enrig
Full names: Deferasirox, Enrig, Deferasirox, Desirox

Indications:
For the treatment of age Beta-thalassemia patients older than 6 years old have chronic iron overload due to frequent blood transfusions (monthly administration of concentrated red blood cells ≥7mL/kg);
For children under 6 years old and iron overload caused by other transfusion-dependent diseases, the safety and effectiveness data of Enrige Chinese patients are limited, and it is recommended to use it with caution based on the specific situation of the patient.

Usage and Dosage:
Starting dose:
The recommended starting daily dose of deferasirox is 20 mg/kg.
For patients who receive monthly transfusions of more than 14 mL/kg of packed red blood cells (i.e., more than 4 units/month in adults) and need to reduce excess iron exposure, a starting dose of 30 mg/kg/day may be considered.
For patients who receive monthly transfusions of less than 7 mL/kg of packed red blood cells (i.e., less than 2 units/month for adults) and who need to maintain iron balance in the body, a starting dose of 10 mg/kg/day may be considered.
Patients who have already responded well to deferoxamine treatment may consider an initial dose of Enriga equal to half the dose of deferoxamine. (For example, a patient receiving deferoxamine 40 mg/kg/day, 5 days a week, or an equivalent dose, may start with 20 mg/kg if switched to Enriga.)

Dosage:
Deferasirox should be taken on an empty stomach at least 30 minutes before a meal, once a day, preferably at the same time every day. Tablets should not be chewed or swallowed whole.

Delasirox should not be taken with aluminum-containing antacids, and the dosage (mg/kg) needs to be calculated and rounded to the nearest whole tablet.
Completely dissolve the tablets in water, apple juice or orange juice (100-200mL) by stirring until a clear suspension is obtained and then drink it. The remaining medicine must be added with a small amount of water, apple juice or orange juice and mixed well before taking it. Not recommended for dissolution in carbonated drinks or milk as it can cause foaming and slow dispersion.

Adverse reactions:
> 10%
Increase in serum creatinine (dose related; 7-38%)
Abdominal pain (21-28%)
Nausea (11-23%)
Vomiting (10-21%)
Diarrhea (12-20%)
Proteinuria (19%)
Fever (19%)
Headache (16%)
Cough (14%)
Nasopharyngitis (13%)
Sore throat (11%)
Flu (11%)
Skin rash (8-11%)

1-10%
Respiratory infection ( 10%)
Bronchitis (9%)
Increased ALT (2-8%)
Joint pain, back pain (6-7%)
Acute tonsillitis (6%)
Rhinitis (6%)
Fatigue (6%)
Ear infection (5 %)
Transaminases (4%)
Urticaria (4%)

<1%
Allergic reactions
Angioedema
Cytopenia, including agranulocytosis, neutropenia and thrombocytopenia; Leukocytoclastic vasculitis

Contraindications:
Allergies
Estimated GFR <40 ml/min/1.73m² or serum creatinine >2 x ULN
Poor performance status and high risk for myelodysplastic syndrome or advanced malignancy
Platelet count <50 x10^9/L

Precautions:
Allergies
Estimated GFR <40 ml/min/1.73m² or serum creatinine > 2 x ULN
Poor performance status and high risk for myelodysplastic syndrome or advanced malignancy
Platelet count <50 x10^9/L

Storage:
Store at room temperature 25°C (77°F); 15-30°C (59-86°F) allowed for short trips
Moisture-proof

Mechanism of action:
DFS has antifungal effects (such as Mucor mold that grows in iron-rich environments), anti-cell proliferation, anti-malarial, anti-oxidative stress damage, anti-cytotoxic induced apoptosis and other pharmacological effects.
1.DFS and Fungal Infection
Iron is a substance that fungi rely on for survival, and it plays a very important role in the growth and toxicity of fungi. The antifungal effect of iron chelators may lie in their removal of iron necessary for fungal growth, thereby achieving the purpose of treating fungal infections.
2.DFS and cell proliferation
Iron plays a very important role in the process of cell proliferation, and iron chelators may have anti-cell proliferation properties. This suggests that iron chelators may become a new type of anti-cancer agent for the treatment of tumors.

Efficacy and safety:
The chemical name of deferasirox is 4-[3,5-bis(2-hydroxyphenyl)-1,2,4-triazol-1-yl]benzoic acid. It is an iron chelator product developed by Novartis Pharmaceuticals in Switzerland. It is the first oral iron-repellent agent approved by the US FDA for routine use. It is approved for children aged ≥2 years. , used in patients with chronic iron overload caused by blood transfusion. In Europe, it is recommended as a first-line drug for thalassemia iron overload patients over 6 years old. Clinical research is currently underway in China; Phase II and III clinical trials and pharmacokinetic studies have shown that it has good safety and tolerability, can significantly reduce iron load in the heart and liver, and is easily accepted by patients. At the same time, it also has pharmaceutical properties such as antifungal (such as Mucor that grows in an iron-rich environment), anti-cell proliferation, anti-malarial, anti-oxidative stress damage, anti-cytotoxic-induced apoptosis, etc.; it can be used for the treatment of secondary hemochromatosis, porphyria cutanea tarda and other diseases.